Acne is an inflammatory skin condition,
commonly affecting the face, chest and back.
It is one of the most common skin conditions,
affecting up to 80-90% of adolescents. Acne
may also occur during infancy due to the
activation of sebaceous glands by maternal
hormones in-utero. It can be a persistent
problem, although it spontaneously resolves
after a period of 4-5 years in about 70%
of people. Approximately 30% of people continue
with their acne into adult life. The incidence
of acne at the age of 40 is reported to
be 1% in men and 5% in women.
Causes
Acne is caused by the overactivity of the
sebaceous (oil) glands. Sebaceous glands
are active due to the hormone testosterone.
Testosterone is produced in men from the
testes and in women from the ovaries and
the adrenal gland. Although testosterone
levels are usually normal in people who
develop acne, the sebaceous glands are overly
sensitive to the hormone. Over production
of oil leads to a greasy feel to the skin.
In addition, there is a change in the growth
of the cells lining the follicular canal
(pore). Instead of dislodging normally and
being carried away by the oil (sebum) when
they die, these cells become sticky and
stick to the inner surface of the gland
and gradually build up a partial blockage.
Symptoms
The partial blockage of the pore is called
a microcomedone and is the precursor of
all inflammatory and non-inflammatory spots
in acne. Bacteria start to multiply within
the blockage, which leads to inflammatory
lesions or red papules. As part of the healing
process, the body recruits white blood cells
which destroy the bacteria but lead to the
formation of pus, leading to comedones (blackheads),
small pimples, large pustules, or painful
swollen cysts. There are many myths surrounding
the causes of acne, which include:
only teenage spots - you'll grow out
of it.
eating too much chocolate and too
many chips.
not keeping your skin clean enough.
Acne can be a devastating psychological disease
commonly affecting the face, neck, chest,
shoulders and back. The latest survey by the
Acne Support Group shows that 12% of people
with acne feel suicidal as a result of having
the condition. Whilst it is a very common
skin condition, it need not be left untreated
and leave a person feeling so desperate that
they want to take their own life.
Treatments
There are many treatments available, which
need to be given at an early stage to prevent
scarring. Treatments are generally longterm,
however with encouragement and support a
person can improve their skin substantially.
First line treatment for acne includes creams
or gels containing the active ingredient
benzoyl peroxide, which is available from
chemists. Salicylic acid is also available
from chemist shops and can be effective
in comedonal (blackhead) type acne. Antibiotics
are a very popular treatment for acne. These
help to reduce inflammation, whilst
killing the bacteria. Other treatments include
preparations containing the hormone therapy
cyproterone acetate (females only).
For those who have not responded to
systemic and topical therapies, isotretinoin,
which is a synthetic vitamin A, can be very
useful. This is a hospital-only drug and
can achieve up to 95% positive results in
patients. There are many potential side
effects from this drug and hence the patient
will need monitoring by their dermatologist.
Generally, treatments need to be taken
for two months before any improvement is
noticed (the ‘two month rule’).
For further information contact:
Acne Support Group,
PO Box 9, Newquay TR9 6WG
Reg. Charity No. 1026654.
The Acne Support Group provides information
and support to those people affected by
acne and those people affected by rosacea.
Information and services available to members
include:
Alopecia is a generic
medical term for all forms of hair loss,
from the patchy baldness of alopecia areata,
which in some cases becomes total loss,
to the diffuse thinning and ‘male pattern
baldness’ of alopecia androgenetica. Hair
loss can be sudden, frightening and severe.
Although it is not life threatening or even
physically painful, alopecia causes a high
level of psychological and emotional suffering.
A survey by Hairline International of women
who had lost, or were losing, their hair
found that 76% felt less of a woman; 40%
said that marriages (or long term partnerships)
had suffered and of these many had broken
up; and 63% said they had been forced to
compromise a career. In many cases, alopecia
patients experience severe depression. A
large proportion of the respondents (43%)
had considered suicide. In addition, patients
often face cruel jokes from others and find
that some healthprofessionals dismiss the
condition as superficial.
Types and Symptoms
Alopecia areata (patchy baldness) affects
men, women and children. It often begins
at puberty. This scalp disease usually starts
with a tiny circumscribed patch of baldness.
Other patches may follow and as one patch
re-grows hair frequently falls out in another.
Alopecia areata frequently spreads very
quickly, sometimes throughout the scalp.
The affected hair follicles slow down production,
become very small and often grow no hair
that is visible above the surface for months
or years. But the follicles normally remain
alive and are ready to resume normal hair
production whenever they receive the appropriate
signal or ‘trigger’. In a third of all cases
patients will have only one small patch
of baldness. The hair re-grows spontaneously
and they never suffer a further episode.
Two thirds of patients suffer the patchy
baldness of alopecia areata throughout their
lives.
Alopecia areata can develop into total loss
of scalp hair (alopecia totalis) or the
loss of all body hair including the scalp
- alopecia universalis - which occurs in
about one fifth of cases.
Alopecia androgenetica (male pattern
baldness) - a large number of women suffer
from thinning hair. In a woman this can
be the female version of alopecia androgenetica,
the natural balding of ageing. It often
occurs after the menopause, but is also
prevalent in younger women who are genetically
predisposed to the condition. It can manifest
itself when triggered by such factors as
eating disorders or an over-sensitivity
to the progestogens contained in some types
of contraceptive pill.
Male pattern baldness often causes a great
deal of distress, particularly in men whose
work brings them into contact with the public.
It can cause a marked fall in self-esteem.
Telogen effluvium - the ‘human moult’
hair loss occurs after the body has suffered
severe trauma. It can occur after a high
fever, childbirth or extreme shock.
Hair loss related to medication -Some
drugs prescribed for other conditions can
cause hair thinning. Including some psychiatric
drugs (eg. anti-depressants) and chemotherapy
treatment.
Self-inflicted hair loss -Trichotillomania
is an obsessional compulsive disorder in
which many patients pull out their own hair.
Causes
Alopecia areata is believed to be an autoimmune
disease in which the body rejects the hair
follicle as foreign. Atopic syndrome often
plays a part and alopecia areata frequently
occurs in patients who have experienced
asthma or eczema from birth. Other factors,
which can be involved in its onset, are
thyroid conditions, anaemia, vaccinations
and stress. It can also be a family problem.
One in five patients can recall a relative
with a similar condition.
Many younger women develop alopecia androgenetica,
because they have an over-sensitivity to
the androgens (male hormones) in the blood.
When they have this pre-disposed sensitivity,
contraceptive pills containing progestogen
can exacerbate the problem. Hormone replacement
therapy (HRT) has similar components to
the pill and many women find that their
hair becomes thinner during this treatment.
The stress of modern life is a common causative
agent.
Treatments
Re-growth success is not always easy to
achieve and doctors are cautious of raising
false hope by offering treatment. For alopecia
areata, and its related conditions, re-growth
success has been achieved by the use of
a combination therapy consisting of topical
steroid creams plus topical minoxidil, systemic
steroids (such as prednisolone) and, occasionally,
zinc. The administration of powerful steroids
can sometimes be enough to 'kick-start'
the hair into re-growth, although doctors
urge caution and careful monitoring for
side-effects. In severe cases and those
of total loss, the phenol derivative diphencyprone
can be successful. In less severe cases
of both Alopecia Androgenetica and Alopecia
Areata, topical minoxidil alone can promote
re-growth. Anti-androgen drugs such as cyproterone
acetate can also help in alopecia androgenetica.
Finasteride, the drug for the treatment
of enlarged male prostate, has recently
been licensed for the treatment of hair
loss in men.
For further information
contact:
Hairline International, The Alopecia Patients’
Society, Lyons Court, 1668 High Street,
Knowle, West Midlands, B93 OLY. The Hair
Trust Reg. Charity No. 1056204. Tel: 01564
775281. Fax: 01564 782270. For information
on membership please enclose an A4 SAE.
Website: www.hairlineinternational.co.uk
Hairline International is a national support
network for those who have lost, or are
losing, their hair and supports all alopecia
patients whatever their type of hair loss.
The organisation:
maintains a network
of contacts to share experiences.
advises on suitable
prostheses and available experts.
Bullous pemphigoid
is a rare, blistering skin disease which
can occur anywhere on the skin, but is more
commonly found on the folds of the skin,
particularly the groin area and the armpits.
The disease is more prevalent in the elderly,
but an increasing number of people are being
diagnosed when in their early fifties. The
average age of onset for the disease is
between 65 and 75. The condition affects
males and females in equal proportion and
is rarely life-threatening. In approximately
30% of people the condition burns itself
out after a number of years, although a
recurrence can occur in later life.
Causes
Bullous pemphigoid is an autoimmune disease,
where an immune response is triggered to
the body’s own skin cells. Separation occurs
between the epithelial and dermal layers
of the skin. The reason for this immune
response remains the subject of research.
The condition is not contagious and may
not be passed on by skin contact.
Symptoms
During a flare-up a non-specific rash first
develops on the folds of the limbs. The
skin becomes inflamed (erythematous) and
very itchy (pruritic). The patient reports
feeling very tired and agitated. The disease
is at times very debilitating and distressing,
with simple tasks becoming a real effort
to perform. The stress of the disease can
exacerbate the condition. These changes
can resemble other skin diseases, such as
urticaria, erythema multiforme and dermatitis
herpetiformis. Consequently GPs often do
not recognise these signs as the early symptoms
of bullous pemphigoid. Following this (prodromal)
phase, fluid-filled blisters develop. A
diagnosis of the condition is confirmed
by examination of a blister by skin biopsy.
The prodromal phase can last from a single
week to several months. Although the condition
sometimes remains localised, it is more
common for dense bullae to progressively
cover both inflamed and normal skin over
the majority of the body area. The skin
feels hot, itchy and very tender. The lesions,
which can be several centimetres in diameter,
are particularly concentrated in body folds.
Lesions may also develop in the mouth and
other mucosal membranes.
Treatments
When a widespread blistering flare-up occurs,
the patient is usually admitted to hospital.
As yet there is no cure for bullous pemphigoid,
but the condition can be controlled using
potent medications. Depending on the severity
of the blistering and the patient’s general
health, it can be treated very successfully
using steroids and/or immunosuppressants.
A large initial dosage of steroids is administered
during a flare-up, in order to bring the
blistering under control. These treatments
can have harmful side effects, and over
the subsequent weeks and months the dosage
is gradually reduced until the lowest efficacious
dose is attained which will control the
condition. A low dosage of the drug treatment
is administered for several months or years
in order to maintain the condition. It is
important to be aware of the risk of infection
to the skin, especially when blisters are
broken. An antibacterial ointment is routinely
applied to the skin for this purpose. In
between flares, although the majority of
people experience no outward evidence of
the disease, it is advisable to keep the
skin well hydrated using emollients (creams,
lotions and bath oils). Some people find
bathing with a non-scented oil very soothing,
whilst others have reported an intolerance
to any bathing. Some people report adverse
reactions to strong sunlight. Although exposure
to ultraviolet (UV) light has not been proven
to precipitate or to exacerbate the condition,
exposing the skin to strong sunlight is
inadvisable as the use of steroid creams
will have thinned the skin, thereby increasing
the risk of skin damage due to sun exposure.
For further information
contact:
Bullous Pemphigoid Support Group, 17 Barley
Mount, Redhills, Exeter, EX4 1RP. The Bullous
Pemphigoid Support Group aims to:
offer support to
people with bullous pemphigoid.
establish commonality between experiences, in order to gain a greater
understanding of the causes of the disease and treatment regimens.
This disease was first
described in 1889 by Jean Darier, a French
dermatologist. It is a rare condition characterised
by itchy, warty bumps often involving the
chest, neck and upper back. The condition
can affect both men and women.
Symptoms
The first signs of the condition usually
appear between the ages of 6 and 20, but
may begin when people are older or, rarely,
younger. The severity of the condition varies
enormously and is unpredictable. Small brownish,
rough topped bumps develop on the skin.
Some patients have scattered spots which
cause very little trouble, but in others
the disease is more pronounced. The chest,
neck or upper back are often involved at
the beginning, but warty bumps may occur
on any part of the body. It is unusual for
people to have much trouble on the face,
except for the skin on the forehead near
the hairline. The scalp and skin around
the ears may be scaly and itchy and most
people notice some small spots in the armpits,
the groin or, in women, under the breasts.
The fingernails are usually affected. They
tend to be rather fragile, split easily
and look as if they have been bitten or
appear dirty. There may be very obvious
long red or white lines running the length
of the nails. Nail changes or flat warts
on the backs of the hands are often present
in childhood, before other symptoms appear.
Pits or small corns occur on the palms of
the hands and less often the soles of the
feet. Occasionally there may be small spots
inside the mouth, which give the roof of
the mouth a rough feeling.
Although the condition is not infectious
or contagious, people with Darier’s disease
show an increased susceptibility to herpes
simplex infections, which exacerbates the
symptoms of the condition. Though the skin
may be clean, affected areas may smell unpleasant,
due to bacterial growth in the rash. The
condition is aggravated in the summer months
due to an inherent photosensitivity, and
through stress.
Causes
It is a dominantly inherited condition.
There is a 1-in-2 chance that each child
of an affected parent will inherit the disease.
The condition is caused by a change (mutation)
in a gene on chromosome 12. This gene makes
a protein found within keratinocytes called
SERCA2. The SERCA2 protein acts as a ‘calcium
pump’.
Cells use calcium to produce signals that
control the complicated ‘machinery’ inside
the cell. In Darier’s disease we believe
signalling is faulty in the skin because
the calcium pumps do not work properly.
This leads to breakdown in the normal bonding
of skin cells. The skin becomes scaly, lumpy
and may blister.
Treatments
Retinoids (Vitamin A derivatives) are taken
orally and improve the overall condition
of the skin, by reducing its lumpiness and
scaling, in most patients. Care must be
exercised when prescribing to sexually active
women, as retinoids could damage an unborn
child. Therefore retinoids are only prescribed
to sexually active women who have been sterilised,
or who are using an effective contraceptive.
Retinoids also cause the drying of lips,
eyes and nose and patients with mild forms
of the disease often decide to live with
the symptoms of the condition rather than
these side effects.
Itching (pruritus) is very common. Emollients
containing an anti-pruritic may relieve
some of the irritation. More severe pruritus
can be controlled with a corticosteroid
cream, containing an antibiotic to prevent
skin infection. As mentioned the affected
skin may smell unpleasant, particularly
in moist areas, due to a bacterial growth
in the rash. This does not mean that the
skin is dirty. Careful washing is important,
and antiseptic solutions for the bath, as
well as creams or antibiotics may help.
The condition can be exacerbated by heat,
sweating and, wool or nylon clothing. Some
patients find that sunlight causes the skin
to flare up, whilst some women find the
condition worsens around the time of their
period.
In a quarter of patients the condition improves
as they get older. Most people lead full
and active lives, with less than a quarter
needing time off work or school because
of the condition.
For further information contact: Darier's
Disease Support Group (DARDIS), PO Box 36,
Milford Haven, Dyfed, SA73 3YF. Tel: 01646
695055.
A Ectodermal Dysplasia
(ED) is not a single disorder, but a group
of closely related conditions of which more
than 150 different syndromes have been identified.
The Ectodermal Dysplasias (EDs) are genetic
disorders affecting the development or function
of the teeth, hair, nails and sweat glands.
Depending on the particular syndrome ED
can also affect the skin, the lens or retina
of the eye, parts of the inner ear, the
development of fingers and toes, the nerves
and other parts of the body.
Causes The ectodermal
dysplasias are caused by altered genes.
The altered genes may be inherited or the
normal genes may become defective (mutate)
at the time of conception. The chances for
parents to have affected children depend
on the type of ED that exists in the family.
It is important to remember that a person
cannot chose or modify the genes that he
or she has, and that events of pregnancy
do not change the genes. Thus, parents who
have a child with ED should not think that
they did anything to cause the defective
gene and should not blame themselves for
its existence. The inheritance patterns
are variable according to the specific type
of ED. Patterns include spontaneous mutations,
autosomal dominant, autosomal recessive,
X-linked dominant and X-linked recessive.
When questions of a diagnosis exist, the
expertise of a geneticist or other doctor
with experience with the EDs is strongly
recommended. Genetic counselling is available
for families.
Symptoms
Each syndrome usually involves a different
combination of symptoms, which can range from
mild to severe, such as: It is important to
remember that not all individuals affected
by the EDs will have physical features that
fit the description of a specific syndrome.
Absence or abnormality
of hair growth
Absence or malformation
of some or all teeth
Inability to perspire,
which causes overheating
Impairment or loss
of hearing or vision
Frequent infections
due to immune system deficiencies or,
in some cases, the inability of cracked
or eroded skin to keep out disease-causing
bacteria
Absence or malformation
of some fingers or toes
Cleft lip and/or
palate
Irregular skin
pigmentation.
In addition to the above they may have:
Cleft lip and/or
palate
Sensitivity to light
Respiratory problems
A lack of breast
development
A host of other
challenges
It is important to
remember that not all individuals affected
by the EDs will have physical features that
fit the description of a specific syndrome.
There may be a great deal of variation in
the physical appearance of the same type
of ED from one affected person to the next.
It is also conceivable for a person to have
a type of ED that has not been described
yet. Nonetheless, the EDs share certain
features, an understanding of which makes
it possible to appreciate the ramifications
for most affected individuals and allows
everyone involved to respond appropriately
to the individual’s needs.
Treatments
Individuals affected by ED face a lifetime
of special needs which may include:
Dentures at a young
age with frequent adjustments and replacements
Special diets to
meet dental/nutritional needs
Air conditioned
environments
Wigs to conceal
hair and scalp conditions
Carrier identification
testing
Protective devices
from direct sunlight
Osseointegrated
dental implants
Respiratory therapies
For further
information contact:
The Ectodermal Dysplasia Society, 108 Charlton
Lane, Cheltenham, Glos. GL53 9EA. Tel: 01242
261332 Reg. Charity no. 1089135 Email:
diana@ectodermaldysplasia.org
obtain answers from
Medical professionals to members’ specific
questions
support families
when they approach organisations such
as Local Authorities, Social Services,
etc., by putting together a personal report
explaining very simply how ED affects
them
liaise with Head
Teachers, Health Authorities and medical
professionals
help families get
the right care for their child in schools,
such as full or part time Carers, fans,
air-conditioning, etc.
help more families
obtain Disability Living Allowance, Disability
Carers Allowance, etc.
support members
in their fundraising
put people in touch
with each other if requested
find pen pals for
the younger members
help families obtain
information regarding ante-natal testing
put the Society
on the databases of Health Authorities,
NHS Trusts, Health organisations, etc.
The Ectodermal Dysplasia
Society has their own Medical Advisory Board
consisting of 12 professionals from various
clinical fields.
The word eczema comes
from the Greek and means ‘to boil over’.
The main features of eczema are dry, itching,
red and inflamed skin. The words eczema
and dermatitis mean the same thing. Eczema
affects about one in every ten people in
the United Kingdom and can be mild, moderate
or severe. Eczema can be a disruptive and
distressing condition and can affect all
areas of personal and family life.
Types, Symptoms
and Causes
Atopic eczema – This is the most common
type of eczema. It usually starts in babies
and young children and is thought to affect
up to one in every five children. The main
features are itching, redness, and inflammation.
Dry, scaling skin is often seen in the creases
of legs, wrists and neck as well as on the
face and forehead. If the skin is weeping
and crusting the skin may be infected.
Atopic eczema is an inherited condition,
linked to asthma and hayfever. It is thought
that people with atopic conditions are sensitive
to things found in their environment (allergens)
which people that are not atopic find harmless.
Allergens may affect the skin by direct
contact, or by being breathed in or swallowed.
Eczema is not contagious – it cannot be
caught from someone else.
Many people have mild to moderate eczema,
which can be successfully managed. However,
some people do have severe eczema, which
may sometimes need hospital treatment. Three
quarters of children with atopic eczema
grow out of it by the time they reach their
teenage years.
Contact dermatitis
– There are two types of contact dermatitis:
allergic and irritant. Both types have similar
symptoms, though the hands are most often
affected. It is sometimes referred to as
occupational dermatitis due to the impact
it can have on a person’s occupation:
Allergic contact
dermatitis – This tends to appear
where the skin is in direct contact with
something, for example, the earlobes in
nickel allergy (if wearing earrings). It
is caused when the immune system overreacts
to a substance that would normally be considered
harmless, and creates an allergic response.
Common allergens include nickel, chromates,
cobalt, rubber, formaldehyde, colophony,
acrylates, epoxy, fragrances, plants, hair
perm solutions, hair bleaching preparations,
flour and garlic. It can be a painful and
disabling condition with skin which is often
dry, red, split, cracked, weeping, fluid-filled
and intensely itchy, sore and stinging.
If the condition is related to the person’s
work, a change of career is sometimes necessary.
Jobs that are at high risk include hairdressing,
catering, cleaning, construction, engineering,
printing, health care, agriculture and horticulture.
Irritant contact
dermatitis – This has virtually
the same signs and symptoms as allergic
contact dermatitis but is caused by repeated
contact with an irritant substance such
as diluted acids, diluted alkalis, solvents,
soaps, detergents, metallic salts, cement,
resins and cutting fluids. The most common
occupations at risk of irritant contact
dermatitis are those that involve wet work,
for example, chefs, bakers, bartenders,
caterers, cleaners, hairdressers, metal
workers, surgical nurses, printers, solderers,
fishermen and construction workers.
Seborrhoeic eczema
– This can occur in adults, children and
babies. In babies it is often associated
with ‘cradle cap’. It usually starts on
the scalp as dandruff that can progress
to redness, irritation and scaling which
can spread to the face and skin creases.
It is a reaction to the increased production
of pityrosporum ovale, a yeast that occurs
normally on the skin in those areas which
generally produce a lot of oil such as scalp,
face and chest and back in men. Candida
(which causes thrush) can also be found
on the skin of people with seborrhoeic eczema
and can make the condition worse.
Gravitational eczema
– Also known as varicose or stasis eczema,
this type appears on the lower legs and
generally affects people in later life,
particularly women. It is related to poor
blood circulation and high blood pressure.
Special care needs to be taken to make sure
that legs are not knocked as the skin can
become thin, fragile, shiny and flaky which
can lead to leg ulcers.
Treatments
The main treatment for eczema is emollients
(moisturisers) and an explanation of the
condition and its treatments. Other treatments
for mild to moderate eczema might include
topical corticosteroids (applied to the
skin), antibiotics, and bandaging. People
with eczema might also be given advice on
how to avoid allergens, the ‘triggers’ that
make their eczema worse. Some people also
find complementary medicines useful to treat
their eczema.
Severe eczema might be treated with stronger
topical corticosteroids, ultra-violet light
therapy, drugs which suppress the immune
system, such as cyclosporin, and oral steroids
taken by mouth. New treatments, known as
topical immunomodulators, such as tacrolimus
and pimecrolimus, are now available for
people with atopic eczema. Tacrolimus is
licenced for patients who do not respond
adequately to topical corticosteroids. Pimecrolimus
is used to try and prevent flares.
For further information
contact:
National Eczema Society, Hill House, Highgate
Hill, London, N19 5NA.
Reg. Charity No. 1009671 A company limited
by guarantee registered in England No. 2685083
Tel: 020 7281 3553 Fax: 020 7281 6395
Ehlers-Danlos Syndrome
(EDS) is a group of heritable disorders
of connective tissue, characterised by skin
extensibility, joint hypermobility and tissue
fragility. There are different types of
EDS and these were re-classified in 1997
into six major types, classified according
to their symptoms and signs with each type
running true in a family. Thus an individual
with one type will not have a child with
a different type. EDS is known to affect
both males and females of all races and
ethnic backgrounds, with an estimated prevalence
of 1 in 5000.
Causes
EDS is caused by a defect in the collagen
(connective tissue), which is the main building
block in the body. Collagen provides strength
for the different parts of the body. Some
types are firm to give support, others are
elastic to allow movement and strength,
and still others resemble glue which bind
proteins together. Consequently, defects
in collagen can produce many problems.
Symptoms
Diagnosis is based on the presenting symptoms
and family history. Diagnosis can be delayed,
or overlooked, in some cases as they do
not fit conveniently into a specific type.
A skin biopsy may be taken to study the
connective tissue. Specific tests are available
for certain types of EDS.
Relating to the
skin Hyperextensibility. Stretchy skin
characterises all EDS except for the Vascular
Type (type lV), which has noticeably translucent
skin with visible veins. When the skin is
over-stretched it still retains normal elastic
recoil and snaps back once released. This
is best tested at the neck, elbows or knees. Fragility. Easy splitting of the
skin is particularly common in Classical
Type (Types l and ll). Gaping, ‘fish-mouth’
or ‘cigarette paper’ scars follow minimal
trauma over sites of bony prominence and,
areas prone to trauma such as the forehead,
chin, elbows, knees and shins. Epicanthic folds. These are additional
symmetrical folds of skin at the inner aspects
of the eyes producing apparent broadening
of the nose. Molluscoid pseudotumours. These are
firm, fibrous lumps measuring up to 2-3
cm that develop over pressure points such
as the elbows and knees. Spheroids. Approximately one third
of affected individuals describe small,
firm nodules like ‘ball-bearings’ just beneath
the skin (subcutis). These consist of fibrotic
and calcified fat, which overlays bony areas
such as the shins. Piezogenic papules. These small,
soft, skin-coloured lumps appear on the
side of the heel when standing and disappear
when the foot is elevated. Although usually
symptom-less they can occasionally be painful. Varicose veins. These are more common
in many types of EDS.
Relating to the joints
Hypermobility is assessed using the Beighton
scale. A score of 5/9 or higher defines
hypermobility.
Dislocation and partial dislocation; this
is common due to unstable joints.
Chronic joint and limb pain. Pain is common
even when skeletal X-rays are normal.
Bruising and haematomas
Easy bruising, at sites of trauma, accompanies
most forms of EDS. This implies increased
fragility of blood capillaries and poor
structural integrity of the skin. When bruising
presents in a child it may be incorrectly
attributed to non-accidental injury.
Mitral valve prolapse
This is quite common and should be diagnosed
by echocardiography, CT scan or magnetic
resonance imaging (MRI).
Less common features Arterial/uterine/intestinal rupture
due to tissue fragility. Hernias are also
relatively common. Scoliosis (curvature of the spine)
may be present at birth or can develop in
later life. Gum disease. Gastrointestinal diverticulae.
Psychological
The main problem with having Ehlers-Danlos
syndrome is that the person can look very
fit and may often not be believed that they
have joint pain and other symptoms. Diagnosis
is often delayed and misdiagnosis is relatively
common. Some forms of EDS may be misdiagnosed
as child abuse/self-inflicted injury. Where
there is severe skin involvement, scarring
can be severe and the person needs to learn
to cope with disfigurement.
Treatment
This depends on the presenting symptoms
but simple precautionary measures will greatly
lessen the chances of accidental trauma,
scarring and bruising. It is important to
carefully balance the advantages of less
frequent injuries and the disadvantages
of over-protection in a child. Simple measures
like padding of the lower legs and elbows
in children may reduce the number of injuries.
Surgery and stitching of skin injuries should
be undertaken with great care as fragile
tissues may tear. Stitches need to be left
in longer than normal. Bracing and splinting
may be used to support unstable joints.
Orthopaedic surgery may be necessary but
is not always successful. Physiotherapy
and occupational therapy advice may be sought
in order to strengthen muscles and to aid
daily living.
The prognosis depends on the specific type
of EDS. Life expectancy can be shortened
in the Vascular Type (type lV) due to the
rupture of vessels and organs. Pregnancy
can be life threatening in the Classical
and Vascular Types (types l,ll,lV).
For
further information contact:
Ehlers-Danlos Support Group PO Box 335,
Farnham, GU10 1XJ.
Epidermolysis bullosa
is the name given to a group of genetically
determined disorders, which are characterised
by an excessive susceptibility of the skin
and mucosae to separate from the underlying
tissues following mechanical trauma. The
individual diseases vary in their impact
from relatively minor disability (e.g. limitation
of walking distance because of blistering
of the feet), to death in infancy. There
are three broad categories of epidermolysis
bullosa: epidermolysis bullosa simplex,
dystrophic epidermolysis bullosa and junctional
epidermolysis bullosa. Within each of these
categories, there are several sub-types
which are clinically, and probably genetically,
distinct.
Types and Causes
EB Simplex - A group of inherited disorders
characterised by mechanically induced blistering
occurring within the epidermis itself, as
a result of lysis of the basal keratinocytes
(breakdown of epidermal skin cells). There
are several established variants, of which
the following are the most important:
Weber-Cockayne
type -epidermolysis bullosa localised
to the hands and feet.
Koebner type
- generalised epidermolysis bullosa simplex.
Dowling-Meara
type - epidermolysis bullosa simplex
herpetiformis.
EB Simplex with
muscular dystrophy.
In addition, there
are a number of rarer variants which are
encountered from time to time. The prevalence
of the different forms of epidermolysis
bullosa simplex can only be estimated. Weber-Cockayne
type is probably 10-20 cases per million
population, Koebner is rarer, possibly about
2 cases per million and Dowling-Meara appears
to be in the region of 5-10 cases per million.
Almost all forms of epidermolysis bullosa
simplex are inherited as autosomal dominant
traits, although some rare forms are inherited
as autosomal recessive traits. People with
the generalised and the localised forms
almost always have extensive family histories
of the condition.
Dystrophic epidermolysis bullosa
- A group of inherited disorders characterised
by mechanically induced blistering occurring
immediately below the lamina densa of the
basement membrane zone. These disorders
derive the name dystrophic from the tendency
of the blisters to heal with atrophic scarring.
Dystrophic epidermolysis bullosa may be
inherited as an autosomal dominant or an
autosomal recessive trait. In general, it
tends to be most severe when inherited as
a recessive, and mildest when
inherited as a dominant, but there is considerable
clinical overlap. There are few data to
indicate the prevalence of dystrophic epidermolysis
bullosa. A recent study in Scotland indicated
an estimated prevalence of 21.4 cases per
million.
Junctional epidermolysis bullosa
- A group of inherited disorders characterised
by mechanically induced blistering occurring
within the basement membrane at the level
of the lamina lucida. There are three broad
types of junctional epidermolysis bullosa:
Herlitz junctional
epidermolysis bullosa - in which death
is probable within the first two years
of life.
Non-Herlitz junctional
epidermolysis bullosa - in which many
people will survive into adult life.
Because most affected
individuals die early in life, the incidence
of junctional epidermolysis bullosa is particularly
difficult to ascertain. It is estimated
that the incidence of new cases is approximately
the same as for dystrophic epidermolysis
bullosa, i.e. around 20 per million births.
To date, all types of junctional epidermolysis
bullosa have been transmitted as autosomal
recessive traits.
Symptoms and Treatments
Due to the large number of different forms
of epidermolysis bullosa with greatly differing
symptoms and prognoses, it is unwise to
try to describe these in any detail here.
Broadly, the common features of the condition
relate to blistering of the skin and mucosae
at the slightest friction. In some forms
the blistering is confined
to particular areas of the body, such as
the hands and feet. In others the blistering
can affect all of the body. The blistered
areas in some forms of epidermolysis bullosa
heal normally, in others healing is accompanied
by scarring.
In view of this, specific information should
always be obtained from a competent clinician
who will be able to relate the information
given to the precise form of epidermolysis
bullosa that the person has. The patient
support group DEBRA can provide the names
of clinicians and other health care professionals
who have a specialist interest in, and knowledge
of, the condition.
For further information contact:
DEBRA, DEBRA House, 13 Wellington Business
Park, Dukes Ride, Crowthorne, Berkshire,
RG45 6LS.
DEBRA is an international not-for-profit
organisation co-ordinating efforts to help
change the lives of people affected by EB.
DEBRA’s main activities are:
funding medical
and scientific research into EB.
providing specialist
advice by a team of nurses and social
workers to people with the condition.
DEBRA produces a
range of information materials for people
whose lives are affected by EB, and a
range of materials for health professionals.
DEBRA runs holiday
homes in Brittany, and in Blackpool and
Weymouth for people with the condition.
Herpes
simplex causes cold sores, whitlows on the
fingers/hands and genital herpes. By adulthood,
herpes simplex infection in the UK is around
70%, worldwide it nears 100%. Herpes simplex
can be differentiated into two genotypes,
type 1 being much more common than type
2. Infection rates are detected by antibody
tests since the majority of people infected
are unaware of it, being asymptomatic. Recurrences
only affect a minority of those infected
and occur due to a lowered immune response
which may be caused by illness, stress,
tiredness, etc. There are nine known human
herpes viruses. Herpes simplex and herpes
varicella-zoster (chickenpox) cause skin
conditions. The other viruses in the herpes
family may cause malaise but do not affect
the skin.
Cause
Herpes simplex virus types 1 and 2 produce
sores: cold sores on the face, herpetic
whitlows on the hands and fingers and “herpes”
elsewhere on the body, especially on the
genitals.
The virus is transmitted by direct skin-to-
skin contact with the affected part. The
incubation period is usually 2-14 days,
however asymptomatic carriers may develop
symptoms years after first contracting it.
Symptoms
The primary illness last about 2-3 weeks
and is self-limiting: flu-like symptoms
followed by blisters (on ordinary skin)
which open into sores or ulcers before scabbing
over and healing. After the primary infection,
herpes simplex virus remains latent in the
body and may reactivate at a later date.
If herpes simplex recurs,
it will only affect the dermatome (nerve
region) where infection took place causing
one or more spots. These may be preceded
by flu-like symptoms. Neuralgia, described
variously as scalded skin, deep aches, tingling
or shooting nerve pains, may be experienced
in the dermatome either before or between
outbreaks. Symptoms are not spread to other
nerve regions. With time recurrences usually
diminish both in frequency and
Treatments
If the primary infection of herpes simplex
is severe, a course of antiviral drugs may
be prescribed: aciclovir, Famvir or Valtrex.
Otherwise all that is necessary is to alleviate
symptoms using antipyretics, analgesics
and topical anaesthetics as appropriate.
Eye infection: Varicella-zoster
virus may affect the ophthalmic division
of the trigeminal nerve causing pain in,
usually, one eye. Diagnosis is by fluoroscein
stain. Treatment with antiviral ophthalmic
cream as well as antiviral pills is recommended.
Eye infection may damage sight or even,
rarely, lead to blindness.
If herpes simplex recurs with a frequency
that the patient finds distressing, antiviral
treatment may be given prophylactically
for a period of six months (minimum useful
treatment).
Self-help is also often effective. The Herpes
Viruses Association can offer suggestions,
as well as counselling, so that the patient’s
anxiety is ameliorated.
Psychological Effects
A diagnosis of herpes simplex can be very
distressing. Since the early 1980s, inaccurate
and deliberately misleading information
has appeared in newspapers and magazines.
Women, in particular, should be reassured
in advance by the diagnosing doctor about
two myths which they may come across: herpes
simplex is not a causal factor for cervical
cancer and women who catch herpes simplex
prior to conception need not assume they
will need a Caesarian section.
A diagnosis of herpes simplex can often
cause greater psychological misery than
physical suffering. Sexually transmitted
conditions carry a stigma, exacerbated in
this country by an immature national attitude
to sex, characterised by prurience and prudery.
The ‘herpes hype’ and stigma associated
with the condition means that most people
remain secretive about the condition.
For further information contact:
Herpes
Viruses Association (SPHERE) 41 North Road
London N7 9DP
The
Herpes Viruses Association and its officially
recognised subgroup the Shingles Support
Society (see “shingles and chickenpox”)
have as their aims:
to promote good health by advancing
information about herpes viruses and the
means by which these conditions may be
most effectively prevented and treated
to the public, media and medical professionals.
to supply specific answers for the public
and members of the association who are
affected by the viruses through the confidential
helpline run by trained volunteers who
all have herpes simplex, letters or counselling.
to provide members with leaflets on
every aspect of “living with herpes”,
leaflets, a lively and informative quarterly
journal as well as workshops, seminars,
local contacts and social events.
“I thought my life
was at an end and I would never have sex
again,” wrote Darren. He is now in a
relationship.
Hidradentis suppurativa
is a disease of the apocrine sweat glands.
It was first diagnosed as a condition by
Velpeau in 1839 and in 1854 was associated
with a peculiar inflammation of the sweat
glands by Verneuil, who also named the disease.
Hidradenitis suppurativa is sometimes referred
to as: apocrinitis, apocrine acne, Verneuil's
disease, Velpeaus disease, Fox-den disease,
acne inversa or hidradenitis axillaris.
Although not a fatal disease the ramifications,
both physical and emotional, that accompany
hidradenitis suppurativa, can prove difficult
to overcome for people struggling with the
condition. It is estimated to affect up
to 3% of the world's population.
Causes
There are many theories about the causal
factors for this condition, however a definitive
cause remains elusive.
Symptoms
The disease affects the areas where the
apocrine glands occur. During the first
few weeks of foetal development these glands
cover the entire body, but have receded
and stabilised by the time of birth into
the pubic regions. Here they stay dormant
until puberty.
The onset of hidradenitis is found most
commonly between the ages of 11 and 30,
although cases have been documented in children
as young as 2 years. Tender red nodules
develop in the apocrine glands of the axillae
(commonly in women), in the groin and on
the buttocks (commonly in men), which are
firm at first, but later become fluctuant,
painful and eventually rupture discharging
pus.
Hidradenitis develops
in three stages and due to its insidious
nature is normally misdiagnosed in its first
stage as a single abscess or boil and is
thus treated as such. It is only when the
disease has reached stage II that the majority
of accurate diagnoses are made.
The spread of the condition
is by a mechanism known as sinus tracting.
This is caused by the effect of bacterial
infection, and pressure from the resulting
abscesses, forcing a pathway under the skin
surface and infecting other tissues of the
body.
Types
Stage I - Single or multiple abscess like
growths in isolated incidences, with no
scarring or sinus tract involvement. The
possibility of long term remission at this
stage is very high and people with the condition
may have periods of up to several years
between outbreaks.
Stage II - Multiple
or single abscess like growths in multiple
regions, with possible sinus tract scarring
beginning to occur. This stage is characterised
by infrequent periods of remission. It is
at this stage that the majority of diagnoses
are made.
Stage III - Multiple
abscess like growths throughout the pubic
areas; widely spread scarring from previous
infections and formation of sinus tracts,
giving rise to constantly weeping open wounds.
Treatments
Hidradenitis suppurativa is not a contagious
disease and cannot be transmitted from person
to person.
There is no cure at
present for the condition. However, if caught
in the first stage, prompt surgical intervention
can offer a good possibility of full remission.
Some treatments have
met with limited success and it is important
to consider possible side effects of any
course of treatment.
For
further information contact:
H.S.S.Group UK & Ireland, 207 Fernhill Road,
Bootle, Merseyside, L20 0AG.
Aneurocutaneous syndrome
of streaky, patchy, whorl-like, or linear
macular hypopigmentation of the skin, often
associated with seizures, developmental
and intellectual retardation, and other
anomalies. Dr Ito first wrote in a Japanese
medical journal in 1952 about some patients
he had seen who had a pattern of skin markings
in a V shape over the spine and in lines
down the arms and legs. Over the years other
doctors have written in medical journals
describing one or two patients with the
same skin markings. Some of the patients
have had problems with development. Until
recently no one knew the cause of Ito’s
disease but the results of research carried
out in Manchester have been confirmed by
other studies in the USA.
Differential Diagnosis:
Ectodermal Dysplasias, especially Naegeli
Type; Incontinentia Pigmenti (Bloch-Sulzberger
Syndrome); Systematized Nevus Depigmentosus;
Tuberous Sclerosis; carbohydrate-deficient
glycoprotein deficiency Type III.
Causes
The body is made up of millions of cells
usually each one has the same genetic instructions
as each other (chromosomes and genes). In
Ito’s disease there are two groups of cells,
one with one set of genetic instructions
and another with a slightly different set
of instructions. It is the upset in “balance”
between the two sets, which causes the problem.
In the type with the
extra chromosomes, usually the egg and the
sperm are normal when they join together.
The fertilised egg then divides and it is
at this division or one of the following
divisions that the chromosomes do not divide
equally giving rise to a set of cells with
an extra chromosome.
In the type with the
gene variation the egg and sperm contain
normal gene instructions and when the fertilised
egg divides a gene fault occurs in one of
the cells and thereafter all the cells resulting
from that cell contain the faulty gene.
The above problems
just happen by chance and are not caused
by anything the parents have done and are
not usually inherited.
Symptoms
These include asymmetry of the body, patchy
pigmentation which can occur on any part
of the body, but not normally the palms,
scalp or soles of the feet. Gross motor
and psychomotor retardation, epilepsy, scoliosis,
dental and bone anomalies, unusual ophthalmic
features and kidney problems, amongst others.
How a child is affected depends partly on
the particular chromosome or gene fault
and partly on how many cells contain the
fault. The sort of problems children with
Ito’s disease have can be divided into two
types: Physical and Developmental. Not all
children have all of the problems.
Physical problems
These include problems obvious at birth
such as unusual shape of joints, extra fingers,
etc. Many children with Ito’s disease have
more growth on one side of the body than
the other making them a little asymmetrical.
The skin patterns are usually first noted
when a child is a few months old.
Developmental problems
Some children with Ito’s disease make very
good progress and others are slow with their
development. There is no way other than
careful follow-up to predict how a particular
child will develop. Even those children
with problems with development do continue
to grow and make progress although slower
than other children. Extra help in nursery
and school may be necessary.
Treatments Since there is no cure for Ito’s disease,
treatment has to be symptomatic. Epilepsy
is treated with drugs (or occasionally surgery);
Behavioural difficulties (including hyperactivity
and autistic features) are often the most
troublesome for families and frequently
require appropriate intervention. Scoliosis
(curvature of the spine) can be helped by
the wearing of a brace and in some cases
surgery. There is no treatment for the depigmentation
markings of the skin
For further information contact:
HITS (UK) Family Support Network (supporting
families affected by the Hypomelanosis of
Ito Syndrome) National Contact: Sandra Field
33 Fernworthy Close, Shiphay, Torquay, Devon
TQ2 7JQ Tel: 01803 401018
London Contact: Terri Grant 07940 114943
Helpline Tuesday evenings 7-9 p.m. only
(UK) time 020 8352 1824 Email: tgrant@uk.ey.com
or tgrant@hitsuk.freeserve.co.uk
Webpage www.e-fervour.com/hits
HITS (UK) is a family support network which
was specifically set up by families to support
other families with this syndrome because
no other support group existed anywhere
else in the world.
HITS (UK) aims to support families by letter,
e-mail, telephone, internet chat room, 3
newsletters a year and by bringing families
together at events to reduce the sense of
isolation often felt.
The Family Support Network’s aim is: To
enrich the lives of families and children
affected by Hypomelanosis of Ito by facilitating
and encouraging communication and linking
families together, and by being a focal
point offering verbal and written support
:
To put families who have a common situation
or difficulty in touch with each other
to enable them to gain mutual support
and friendship.
To reduce the sense of isolation families
and children with disabilities or special
needs often feel.
To liaise with the medical profession
to communicate the group’s existence and
to share medical information available
to us.
To have quarterly conference calls and
to produce a quarterly Newsletter.
To organise an Annual or Bi-Annual Family
Event.
To promote the importance of the group
and the need for communication.
To organise medical training awareness
days in an attempt to gain earlier diagnosis.
Ichthyosis is a term
used to describe continual scaling of the
skin. It comes from a Greek word ‘ichthys’
which means fish. It is quite rare and can
be inherited (genetic or congenital) or
it can develop later in life (acquired).
The inherited forms of ichthyosis are usually
evident at, or soon after, birth and they
tend to persist throughout life, although
some types improve with age. Most, if not
all, of the skin is affected. Ichthyosis
can cause severe psychological problems.
The public’s reaction to its appearance
is often hostile and unsympathetic. Staring
and teasing are common.
Causes Faulty copies
of genes, which are passed on from one generation
to the next, cause inherited ichthyosis.
Each type of ichthyosis is due to a different
genetic mutation and the pattern of inheritance
varies. Recent research on bullous ichthyosis
has led to the discovery of a particular
mutation to the gene that controls the skin
protein keratin. Other types of ichthyosis
have been shown to be related to abnormalities
in the lipid or fat chemicals in the skin
such as in X-linked ichthyosis.
Symptoms
These can vary greatly between the different
types of ichthyosis (see individual types).
Darkened and rough skin can appear as scales
separated by deep creases. In some cases
skin blisters and peels. The main forms
of inherited ichthyosis are as follows:
Ichthyosis vulgaris
- The most common form and usually quite
mild, with fine scaling or roughness on
the arms and legs.
X-linked recessive
ichthyosis - Occurs in men and boys
and causes brownish flat scales mostly to
the arms, legs, and tummy. Varies in severity
and some affected babies have a difficult
delivery. Genetically, it is passed on through
the female line.
Non-bullous ichthyosiform
erythroderma and lamellar ichthyosis -
These are very rare, are often severe, with
prominent scaling, and cause redness over
most of the skin. Affected babies are often
born with a shiny waxy second skin (collodian
membrane), which sheds in a few days.
Bullous ichthyosis
- This is another rare red ichthyosis, which
causes blistering, fragile skin early in
infancy and thick scaling especially around
the joints later on.
Harlequin ichthyosis
- This condition is an extremely rare but
very severe form of ichthyosis, which causes
thick plates of scale and severe complications
at birth. Many affected babies do not survive.
Netherton’s syndrome
- The affected infant has red inflamed scaly
skin from birth, fragile spiky hair and
difficulty gaining weight in early childhood.
Sjogren–Larsson
syndrome - Another condition that also
produces scaling and thickening of the skin
which may not be obvious until a few months
of age. Affected babies also have a type
of cerebral palsy.
Conradi-Hunermann
syndrome, neutral lipid storage disease
and KID syndrome - These are very rare
and are associated with other medical problems.
Treatments
The milder ichthyoses can be well controlled
using regular moisturisers (emollients),
including bath oils, soap substitutes and
moisturising creams. The more severe forms
may make affected babies quite unwell and
treatment in special care baby units will
give them a good start. Moisturisers are
again important as an ongoing treatment
and antibiotics may be needed from time
to time. A group of drugs called retinoids
may be tried as they lessen the scaling
in many forms of ichthyosis. The complicated
varieties of ichthyosis will need additional
treatments according to their severity.
As yet there is no cure for ichthyosis.
For further information
contact:
Ichthyosis Support Group (ISG)
People with ichthyosis and carers: ISG National
Contact, 16 Cambridge Court, Cambridge Avenue,
Kilburn, London, NW6 5AB.
Tel/Fax: 020 7461 9034 (after 8pm).
Medical professionals:
ISG Secretary, 2 Copnor, Woolton Hill, Nr.
Newbury, Berkshire, RG20 9XH. Tel: 01635
253829.
Leg ulceration is a
chronic disease occurring predominantly
in the older population. An ulcer can be
defined as a local deficit or excavation
of the surface of an organ or tissue, which
is produced by the sloughing of inflammatory
necrotic tissue. Ulcers may be present for
many years but can be successfully treated
following a comprehensive medical assessment,
appropriate clinical management and patient
compliance to treatment. Estimates of prevalence
range from 1.5 to 1.8 per 1000 total population,
rising to 3 per 1000 at age 61-70 and to
20 per 1000 in people aged 80 and over.
The annual cost to the National Health Service
(NHS) is estimated to be in the region of
£300-600 million. The majority of patients
are cared for bycommunity services.
Venous ulcers account
for 70-90% of all cases. Arterial ulcers
account for 5- 20% of cases. Combined venous
hypertension and arterial disease account
for 10-15% of cases. Less common causes
account for 5-10% of cases and include:
diabetes, vasculitis, neoplasm, infection
and trauma and other less specific causes.
Causes and Symptoms
Venous ulceration can result from chronic
venous hypertension in the lower limb, usually
due to malfunctioning valves in the leg
veins, of either congenital or acquired
origin, resulting in a backflow of blood.
The superficial venous network is exposed
to higher pressures than normal resulting
in oedema, capillary damage and thinning
of the dermis (inner layer of the skin containing
vessels, nerves and fibrous tissue - covered
by the epidermis). The subsequent leakage
of red blood cells and large protein molecules
creates the physiological conditions which
can lead to ulcer formation. Varicosities
(abnormal swelling of veins) are commonly
present. Often the lower limb is swollen
with a brown discoloration of the skin and
the presence of an irritating eczema.
Arterial ulcers result
from a reduced arterial circulation. Common
causes include atherosclerotic changes in
the main vessels and small emboli (blockages),
not uncommonly the result of hypertension.
Smoking and poor diet are contributory factors.
The lower limb may appear pale or a dusky
red colour and the patient commonly complains
of pain, particularly when the limb is elevated
or during exercise.
Leg ulceration impacts
on the quality of daily life through pain,
physical restrictions, sleep disturbance,
reduced energy, emotional reactions and
social isolation.
Treatments Venous ulceration - treatment aims
to counteract the high pressure in the superficial
veins, increase the calf muscle pump mechanism
to assist venous return and reduce oedema.
This is achieved by applying graduated compression
from the toes to the knee. Local wound management
aims to achieve optimum healing conditions
and avoid the exacerbation of varicose eczema.
Underlying nutritional deficiencies should
be corrected. The prognosis is good, providing
the cause of the ulcer has been correctly
assessed and management decisions have involved
patient consultation. Support or compression
hosiery is often necessary to prevent recurrence
following healing.
Arterial ulceration
- treatment includes the local management
of the wound and avoids compression, which
would exacerbate the arterial insufficiency.
Vasodilator drugs may be used. The prognosis
is poor, unless surgical intervention is
possible to correct the arterial problems.
Cessation of smoking, weight control, a
nutritious diet and maintaining mobility
are beneficial.
PRESSURE ULCERS
A pressure ulcer is
an area of damaged skin and tissue caused
by unrelieved pressure, shear or friction,
typically sustained during long periods
of bed or chair rest during illness or disability.
Prevalence rates are estimated at between
7% and 10% in hospital and community patients.
It is suggested to be costing the NHS in
excess of £400 million per year. Treatment
is aimed towards prevention, including relief
of the pressure and avoidance of friction
and shear forces. There are many pressure
relieving aids available and methods of
care aimed at prevention. Damaged skin should
be covered with a suitable dressing.
For further information
contact:
Tissue Viability Society, Glanville Centre,
Salisbury District Hospital, Salisbury,
SP2 8BJ.
The Tissue Viability
Society is concerned with the development
of good practice in the management of wounds
of all types, particularly chronic wounds
such as pressure ulcers and leg ulcers.
The society does so by:
maintaining a multi-disciplinary network
of health care professionals to share
and disseminate information.
providing education through conferences
and study days.
publishing a quarterly Journal of Tissue
Viability.
providing a range of information booklets
to the public and professionals.
Wound Care Society,
PO Box 170, Hartford, Huntingdon, PE29 1PL.
Lichen sclerosus is
a chronic inflammatory skin condition resulting
in well-defined shiny white spots on the
skin. The condition can be seen in all ages
but occurs more frequently in women of middle
age. The condition more commonly affects
women than men.
Symptoms
Lichen sclerosus most commonly affects the
genital area. The predominant symptom is
acute (intractable) itching of the vulval
area. There may also be soreness and pain.
Blood blisters and sores can occur, especially
where the skin is scratched. The vulva thickens
and looks white. Scarring may occur. Splitting
of the skin can occur in the vulval and
perianal area, especially on passing bowel
movements.
Atrophy and change
in the vulval features occur in long established
cases and include fusion of the labia over
the urethra, causing difficulty in urination.
The clitoris may become ‘buried’. There
may be narrowing of the vaginal opening
making sexual intercourse difficult. Infections
(including ‘thrush’) may be present.
In men the penis is
affected by itching, soreness, difficulty
in retracting the foreskin and, due to a
narrowing of the urethra, in urinating.
The skin may look pale.
There is a small risk
of cancer currently estimated at around
3%. The condition can also affect other
areas of the body, for example the shoulders.
Children are similarly affected.
Psychological
One of the main problems is recognition
and appropriate diagnosis by the General
Practitioner. Misdiagnose of the condition
as ‘thrush’ is relatively common, or as
a psychological complaint to be treated
with anti-depressants and tranquillisers.
An accurate diagnosis is delayed for many
years in some people. Earlier specialist
referral would reduce the distress caused
to people who fail to respond to treatments
for infections.
There is a tendency
for those experiencing painful sex to have
a sense of low confidence and self worth,
to feel they are freaks, to experience a
loss of femininity/masculinity. Many grieve
for their sexuality. There is often a very
real feeling of total isolation and despair.
Patients have difficulty in coming to terms
with the disabling effect lichen sclerosus
can have on everyday life.
Treatments There is,
as yet, no cure for lichen sclerosus.
Treatments aim to relieve
the symptoms of the condition by the use
of topical steroid creams. Surgery is not
part of the treatment but is sometimes necessary
to separate fused labia and to relieve the
symptoms of scarring.
For further information
contact:
National Lichen Sclerosus Support Group,
PO Box 7600 Hungerford Berkshire, RG17 7XD
(enclose SAE for reply).
Lupus is an autoimmune
disease, a type of self-allergy whereby
the patient's immune system overproduces
antibodies which then attack the person's
own tissues. Lupus is neither infectious
nor contagious, and its effect is inflammatory.
Patients principally suffer extreme fatigue
and joint and muscle pains. A variety of
other symptoms are possible, as well as
damage to vital organs if the illness has
taken a strong hold. Nine out of ten people
with lupus are female, and whilst it is
usually triggered between the ages of 15
and 50, children can also have the illness.
Some 50,000 people in the UK are thought
to have lupus, many yet to be diagnosed,
with the incidence of the disease being
greater in Afro-Caribbean and Asian communities.
Causes
The causes of lupus are not fully understood,
although heredity, puberty, hormonal activity
and change, childbirth, viral infections,
sunlight, the menopause, trauma or strong
medication may play a part in triggering
the illness. Lupus can affect people of
all ages and both sexes. The predominance
of the illness seen in females is due to
the influence of hormonal factors.
Symptoms
Lupus can present in a complex number of
ways, even to the extent of 'mimicking'
other illnesses such as rheumatoid arthritis,
multiple sclerosis (MS) and myalgic encephalomyelitis
(ME). Lupus can be hard to diagnose and
the condition can be overlooked, sometimes
for years, unless the physician is alert
to its possibility. Many patients differ
in symptom patterns, which are taken into
account with specific blood tests prior
to diagnosis.
A skin problem does not necessarily occur
in systemic lupus (SLE) but is always present
in discoid lupus (DLE), where rashes occurring
on the face, scalp or neck can be quite
severe. Both types arise from a disorder
of the immune system. DLE can develop into
systemic lupus, but does not always do so.
It is most unusual for the SLE patient to
develop DLE. In summary:
SLE - acute, subacute or chronic
skin rashes may occur, mainly on the arms
and upper body, together with the possibility
of a 'butterfly' rash on the cheeks.
DLE - disc-shaped lesions can occur
on the face, neck or scalp.
Lupus is a weighty burden for the patient,
being incurable and causing physical limitations
and pain. Continuous resolve is needed by
patients and their families to ensure that
they are not 'dragged down' by lupus and
are able to restore and maintain some quality
to their lives.
Treatments
Both SLE and DLE skin conditions respond
to treatment, but may recur where the underlying
illness flares from time to time. The reasons
for flare-ups are not always well understood.
There is, as yet, no cure. Careful monitoring
of the illness together with a flexible
treatment programme, enables the condition
to be controlled in the majority of patients.
Treatments may range from no medication
at all in very mild lupus, through NSAIDs
(non-steroidal anti-inflammatory drugs)
and corticosteroids to immunosuppressives
and even stronger medication, where the
illness is more serious. As the patient's
lupus becomes better managed, the physician
will try to reduce the volumes and/or levels
of medication, eventually to reach the lowest
possible maintenance level. Patients can
learn to reduce the impact of the illness
by becoming better educated about the condition,
by learning to 'pace' their daily routines,
by taking regular rest and by reducing the
incidence of stress, depression, anger and
pain wherever possible. For many lupus patients,
lifelong medication and care is needed,
yet in a smaller percentage the illness
has been known to recede, particularly after
the menopause. Physicians now have much
greater knowledge of this still-mysterious
disease and have a wider range of drugs
at their disposal, which have made for easier
control of the individual’s own ‘brand’
of lupus.
Good information is
also available to patients through lupus
consultants, specialist nurses and support
groups.
For further information
contact:
LUPUS UK, St James House, Eastern Road,
Romford, Essex, RMI 3NH.
Lymphoedema creates
distressing, debilitating and uncomfortable
swelling. Lymphoedema most commonly affects
a limb but the face, neck, abdomen, genitals
and internal organs can be involved. It
is still not a well-documented condition,
but early diagnosis and treatment are essential
for the optimum management of the disorder.
Symptoms, Types
and Causes
Lymphoedema is a serious disorder for which
there is no cure and which, without the
appropriate treatment can gradually deteriorate.
A worsening of the condition is not limited
to just the increase in size of the limb
but also to the insidious changes in tissue
structure, e.g. elephantiasis, and susceptibility
to frequent acute inflammatory episodes
(infections) with the resultant long-term
dependency on antibiotics.
The physical and psychological
impact on the patient with a swollen limb
is immense. Extreme discomfort, heaviness
in the affected part and problems of movement
can be experienced. Over time, there may
be a gradual thickening of the skin at the
affected site and spontaneous lymphorrhea
can occur. Repeated acute inflammatory episodes
sometimes resulting in hospitalisation are
not unknown. The quality of life is greatly
diminished. Clothes and shoes that fit are
difficult to find. The person has to adapt
to an altered and more limited daily lifestyle.
There is reduced working capability, intolerance
to heat (climatic or in a sauna or baths
for example) and minimal participation in
social activities.
Primary lymphoedema
- is due to a fault within the lymph conducting
pathways, i.e. no identifiable cause. It
is usually determined from birth and may
be due to malformation of the lymphatic
system. Swelling develops progressively
at different stages in life and presents
usually in the lower limbs. It is normally
indolent but associated discomfort and debility
are very common. Congenital lymphoedema
can also be associated with numerous developmental
abnormalities. There are several different
types of primary lymphoedema:
Nonne-Milroy disease - congenital
familial lymphoedema presenting at birth
or soon thereafter. Any part of the body
may be affected.
Lymphoedema Praecox - onset is
usually during puberty and represents
some 80% of all primary lymphoedemas,
usually affecting the lower limbs.
Lymphoedema Tardum - onset is after
35 years of age.
Secondary lymphoedema
- is when the cause of lymphatic system
failure is known, e.g. surgery, radiotherapy,
infection, trauma such as severe injury,
burns or perhaps thrombosis. The progression
of lymphoedema can be classified in three
stages:
the reversible stage when swelling
is soft and will pit easily with light
finger pressure. The lymphoedema will
disappear with bed rest.
the spontaneous irreversible stage
when the swelling hardens, pitting is
not so obvious and there is the slow formation
of fibrous tissues (fibrosis) where protein-rich
fluid has collected.
the elephantiasis stage when
there is extreme swelling and thickening
of the skin.
Treatments
Drug therapy is of limited value but diuretics
are still recommended to many patients.
These, in fact, have little or no effect
on lymphoedema and can cause long-term problems.
Pressure pumps are contra-indicated except
under strict medical control. Surgery, such
as 'debulking' or 'reduction' is a procedure
of last resort. A person with lymphoedema,
or at risk from lymphoedema following surgery,
should never allow blood to be drawn, blood
pressure to be taken or injections given
in the affected limb or body part as these
procedures may well manifest or exacerbate
the condition.
Lymphoedema cannot be cured but it can be
controlled with early diagnosis, correct
and early treatment and ongoing management.
Decongestive Lymphatic Therapy (DLT) otherwise
known as Complex Decongestive Therapy (CDT)
has been the recommended treatment for lymphoedema
for many years. Initially, an intensive
course of treatment can last from ten days
to six weeks. Following a course of treatment,
compression garments will usually have to
be worn indefinitely to maintain the volume
reduction and tissue improvement achieved.
It is also necessary to ensure that the
body weight remains, or is brought within,
normal limits. After six months to a year,
a repeated course of intensive treatment
may be necessary. In the meantime, daily
exercises and, in an ideal world, regular
manual lymphatic drainage therapy should
be recommended.
Patient compliance
has to be 100% in following the medical
recommendations on a daily basis between
treatments and often the patient will have
to reconsider their work and social lifestyles
in order to comply. The overall benefit
is that the lymphoedematous region is returned
to as near normal as possible. The patient
can enjoy an improvement in their quality
of life and there are fewer, if any, episodes
of cellulitis or other life-threatening
infections.
For further information
contact:
Lymphoedema Support Network, St Luke's Crypt,
Sydney Street, London, SW3 6NH. Reg. Charity
No. 1018749. Tel: 020 7351 4480 (answerphone
out of hours). Fax: 020 7349 9809. Email:
ADMINLSN@lymphoedema.
freeserve.co.uk
Website: http://www.lymphoedema.org/lsn
Available to members:
quarterly newsletter - LymphLine.
up-to-date national/international information
on lymphoedema treatment.
talks from medical experts.
bi-monthly group meetings at the Royal
Marsden Hospital, Chelsea.
general leaflets and reading list.
For professional
enquiries only, contact:
British Lymphology Society, 1 Webb’s Court,
Buckhurst Avenue, Sevenoaks, Kent, TN13
1LZ
Reg. Charity No. 1042561.
Tel: 01732 740850 (answerphone out of hours).
Fax: 01732 459225.
Website:
www.lymphoedema.org/bls
Email:
admin@blsac.demon.co.uk
Neurofibromatosis is
a genetic disorder of the nerve tissue presenting
in two forms. Type 1 (Nf1) affects one in
every 2,500 people world-wide. This equates
to more than 23,000 people in the UK. An
affected person of either sex has a 1-in-2
chance of passing the defective gene on
to any, or all, of their children. However,
everyone is at risk because 50% of cases
result from spontaneous mutation in families
with no previous history of neurofibromatosis.
Type 2 (Nf2) affects one in every 35,000
people world-wide (more than 1,500 in the
UK) and, as with Nf1, an affected person
has a 1-in-2 chance of passing on the defective
gene to any of their children.
Symptoms Type 1 (Nf1) - Six or more coffee
coloured (cafe-au-lait) marks on the skin
in the first five years of life and/or nodules
(lumps and bumps) on or just below the surface
of the skin. Complications include:
learning difficulties
behavioural problems
high blood pressure (hypertension)
curvature of the spine (scoliosis)
malformation of the long bones (below
the knee and below the elbow - pseudarthrosis)
large benign skin tumours (plexiform
neurofibroma)
tumours on the nerves of sight (optic
glioma)
internal, spinal and brain tumours -usually
benign
speech problems
increased risk of epilepsy
hearing defects
can lead to paralysis
can cause premature death
Type 2 (Nf2) - The chief characteristic
of Nf2 are bi-lateral acoustic neuromas (tumours
on both nerves of hearing). Unless there is
a family history of Nf2, diagnosis is often
very difficult. With no outward signs, in
most instances substantial hearing loss has
occurred before Nf2 is suspected. Surgery
is often the only treatment and facial paralysis
is, in many cases, unavoidable. Nf2 is always
serious. Complications include:
benign brain tumours (meningiomas)
tumours of the spine (meningiomas, schwannomas)
cataracts
skin tumours (schwannomas)
can lead to paralysis
can cause premature death
Treatments
In every case, prognosis of Nf1 is uncertain;
no two cases are the same. One of the particular
problems is that the course of the disease
cannot be predicted even within families;
the disease complications do not breed true.
The morbidity and mortality of the disease
is largely determined by which complications
develop. Recent studies, however, have drawn
attention to the significant morbidity in
terms of psychological well-being caused
by the cutaneous neurofibromas themselves.
There is no actual preventative treatment
for any of the disease features. Treatment
for many of the complications is the same
as when they occur in isolation in the general
population. With regard to neurofibroma
removal, there seems to be no particular
benefit derived from either surgical or
laser treatment. Nf2 is consistently a more
severe problem than Nf1. Nearly all affected
individuals eventually develop bilateral
acoustic neuromas which are very difficult
to treat successfully. There is no useful
medical treatment for the tumours at the
moment, all of which need to be removed
surgically. Patients need to be referred
to centres with established expertise in
acoustic neuroma surgery.
For further information contact:
Neurofibromatosis Association, 82 London
Road, Kingston on Thames, Surrey, KT2 6PX.
The Neurofibromatosis
Association is a national charity which
has three main areas of work:
building up a national network of Support
Co-ordinators mainly based at Regional
Genetic Centres; there are currently 12
plus one national Nf2 Co-ordinator. They
can give real support to families affected
with neurofibromatosis and their work
can assist GPs in the management of cases
of the disorder.
providing information for patients,
the public and medical and educational
professionals. The Association is the
only source of accessible information.
It produces a quarterly newsletter, books,
leaflets and videos.
raising funds for research and, through
a Medical Research Board, directing those
funds to the best scientific advantage.
Pemphigus
vulgaris (PV) is one of a group of chronic,
relapsing auto-immune diseases causing blistering
of the skin and mucosal membranes. In pemphigus
disorders the immune system perceives the
skin and mucous membranes as foreign and
an immune response is triggered. Although
the condition is controllable with heavy
immunosuppressive treatment there is no
cure available and, if left untreated, the
disease can prove fatal. Onset usually occurs
in middle age (4th to 6th decades). There
are no definitive statistics but worldwide
incidence of all types of pemphigus is thought
to be between 1 and 5 people per hundred
thousand affected. PV is very rare, with
most General Practitioners never encountering
the disease, which can lead to mis-diagnosis
of the condition.
Causes
Research studies show a genetic predisposition
to the disease, but although researchers
have identified a variety of possible triggers,
these remain speculative. The possible triggers
suggested, include: D-penicillamine derived
drugs, some viruses e.g. Epstein-Barr and
herpes simplex, some food groups particularly
the allium family, exposure to the sun,
environmental pollutants and long-term stress.
Affected individuals
have high concentrations of antibodies produced
by the immune system. These bind to a specific
protein in the skin (desmoglein 3), causing
interference with the skin’s normal function
and separation of epidermal cells. This
often occurs first in the mouth, but lesions
can cover a significant area of skin. The
disease is non-infectious.
Symptoms
Someone with PV may feel ill a lot of the
time, even if the disease is under control.
A patient can be either in remission or
have the condition under control, but still
experience some blistering lesions. These
burn-like lesions often first occur in the
mouth and throat, are extremely painful,
and are frequently mis-diagnosed as ulcers.
The sensation has been compared to having
a candle burning in the throat. Consequently
pain control is a crucial issue. Lesions
on the skin sometimes hurt, and even if
they are not painful, they often itch and
burn continuously, which in itself is physically
upsetting for the patient. The pain endured
by people with the condition can be very
distressing, not only for the person concerned
but also for carers.
Some people manage
the high levels of corticosteroid and immunosuppressive
drug therapies without difficulty, whilst
others find that, apart from long-term side-effects
(eg. osteoporosis), the daily consequences
of using such drugs produce health problems
which are difficult to live with. Due to
its rarity, people often find it hard to
get accurate and up-to-date information
on the condition and most people feel extremely
isolated.
Treatments
The standard treatment is with corticosteroids,
which are often started at high doses (eg.
60-100mg per day), together with immunosuppressive
drugs (Azathioprine, Cyclophosphamide, Mycophenolate
mofetil, Cyclosporin). Other drugs that
are used with varying effects are: Methotrexate;
Dapsone; Gold injections; Tetracycline,
minocycline or doxycycline combined with
niacinamide . Drug doses are reduced slowly
to the minimum required to maintain the
condition under control (which varies from
individual to individual). Topical therapies
are sometimes given in conjunction with
systemic drugs to help heal recalcitrant
erosions. In the pre-corticosteroid era,
PV was generally fatal. Even with steroids
and immunosuppressives, it remains a difficult
disease to treat and has a mortality rate
of about 6%.
For further information
contact:
Pemphigus Vulgaris Network, Flat C, 26 St
Germans Road, London, SE23 1RJ.
As the group has no
funding please could you enclose a stamped
addressed envelope.
The Pemphigus Vulgaris
Network offers general support and:
provides a forum for people living with
PV and those personally or professionally
concerned with it.
gives people an opportunity to contact
other people with PV.
helps people find information they need.
The
Network also offers support to people living
with Mucous Membrane Pemphigoid (including
Ocular Pemphigoid).
The
PV Network is on the British Association
of Dermatologists’ register of support groups
and is affiliated to the International Pemphigus
Foundation (The Atrium Plaza, Suite 210,
828 San Pablo Avenue, Albany, CA, 94706,
USA. Tel: 001-510-527-4970. Fax: 001-510-527-8497.
E-mail: pemphigus@pemphigus.org
The
Network shares a website with the International
Pemphigus Foundation (http://www.pemphigus.org).
Pseudoxanthoma Elasticum
(PXE) is an inherited disorder in which
elastic fibres, which are normally found
in the skin, the retina of the eyes and
the cardiovascular system become slowly
calcified, producing characteristic changes
in these three areas. It is estimated that
about 1 in 70,000 people in the world has
PXE and for no known reason the condition
affects twice as many females as males.
The average age of onset is about 13 years
with a range from 2 to 20 years. The cause
of the disease is unknown.
Symptoms
The first manifestation, and the one which
nearly always makes the diagnosis possible,
is the appearance of highly characteristic,
slightly thickened, patches of skin. The
skin changes seen in PXE are variable. Some
individuals experience very little change,
whilst in others the small 2-5 mm yellowish
or yellow-orange papules may form groups
or coalesce into larger plaques giving a
cobblestone or plucked chicken appearance.
The skin may become lax or folded. The appearance
and location of skin lesions can cause psychological
distress. They most commonly first appear
on the sides of the neck, which leads to
younger persons being accused of not having
washed properly.
Lesions later appear
in other flexural areas, for example under
the arms, folds of the arms, the groin and
behind the knees. The diagnosis can be confirmed
by a skin biopsy, a procedure in which a
small piece of skin is removed for analysis.
In PXE, the biopsy will show a distinctive
calcification of the elastic fibres.
Widespread involvement
of the body is rare. In some cases the onset
of skin lesions may be delayed until late
in life, but many such individuals probably
had small skin lesions for many years which
were unrecognised as PXE. In a few individuals,
suspected of having PXE, the skin involvement
is so mild it may be thought to be lacking,
but very careful examination, or a skin
biopsy, will confirm a diagnosis.
Involvement of other
systems: As has already been mentioned,
the effects of PXE can go far beyond those
affecting the skin, which are the most characteristic
effects of PXE. The ocular and cardiovascular
manifestations are responsible for the morbidity
of the disease. The eyes of 60% of those
with PXE will be affected eventually, usually
in middle life. A smaller number will suffer
from cardiovascular problems affecting the
heart and legs (intermittent claudication),
and from gastrointestinal bleeding.
Treatments
Skin lesions are asymptomatic, causing no
medical problems in themselves, but can
be cosmetically objectionable if they become
severe. There is no specific treatment to
change the appearance of the skin lesions
and skin folds, other than plastic surgery.
Good cosmetic results have been achieved
on the neck, however plastic surgery on
the axillae is more difficult. Some improvement
is possible if the skin sags excessively
in these areas.
Attention to a proper,
balanced diet and control of calcium intake
will help slow down the process which affects
the skin. Calcium is needed for life and
a normal physiology, but not in excessive
amounts. A calcium intake of no more than
800mg per day is recommended. If necessary
a dietitian should be consulted to help
maintain a balanced diet.
Research
The current research in all hereditary disorders
is to look for the defective gene responsible
for the condition and then attempt to add
or replace the missing or altered substance.
In May 2000, the good news that the PXE
gene had been isolated was sent round the
world. The gene has been identified as 'ABC-C6'.
Researchers are now addressing the question
of how and why a loss of function of the
ABC-6 leads to PXE. The answer to this will
hold important clues towards treating and/or
preventing the illness.
For further information
contact:
Pseudoxanthoma Elasticum Support Group (PiXiE),
15 Mead Close, Marlow, Bucks. SL7 1HR.
There are several forms
of psoriasis, which usually appear as patches
of silvery scales on top of areas of crimson
skin. The scales are easily shed or scratched
off. It is a distressing condition, which
can lead to a reduced self-esteem. Over
a million people in the United Kingdom and
Ireland express the condition, equating
to 2% of the population; with men and women
being equally affected. Psoriasis usually
occurs between the ages of 10 and 45 years,
although there are exceptions. It is an
inherited condition, which does not necessarily
recur in successive generations.
Causes
Psoriasis is caused by the over production
of skin cells. The development of skin cells
to replace those naturally sloughed off
occurs at up to seven times the normal replacement
rate. Hence, raised red patches are produced.
These can be covered with scaly, dead skin.
Trigger factors for a psoriasis flare-up
include: infection; damage to the skin (burns,
sunburn and scratching for example) and,
certain medications (eg. anti-malarial,
anti-depressant and beta receptor blocking
drugs). Very commonly, stressful events
such as death, divorce, examinations and
work pressures may precipitate a flare-up
or may exacerbate a mild flare-up. In women,
hormonal changes can affect the condition.
The basic cause of the condition remains
unknown and is subject to ongoing research.
Types and Symptoms Chronic plaque psoriasis (psoriasis vulgaris)
- This is the most common type, appearing
on elbows and knees, or sometimes more extensively
over the trunk and limbs.
Pustular psoriasis
(palmar plantar) - Pustules on the soles
of the feet and palms of the hand, which
go brown and develop scales. The skin often
cracks. More often seen in middle age.
Flexural psoriasis
- This type appears in the armpits, groin
and under the breasts. It is fiery, shiny
red, with little or no scaling.
Guttate psoriasis
- This is quite common in children and teenagers,
often occurring after a streptococcal throat
infection. Lesions appear as small ‘raindrop’
patches.
Psoriasis of the scalp and nails
-Scalp psoriasis affects the majority of
people who develop the condition. Scaling
occurs, especially around the hairline.
It is often itchy and can sometimes lead
to a temporary loss of hair. Nail involvement
is less common. Nails may show pitting,
flaking and ridges. This can be an early
indication of psoriatic arthritis (see
section on Psoriatic Arthritis).
The lowering of self-esteem is one of the
most profound aspects of psoriasis. With
such an emphasis on appearance in society,
the incidence of psoriasis can cause a great
deal of distress. One of the most difficult
aspects is its effects on everyday life.
A reduced self-esteem can affect the freedom
to sunbathe or swim, visit the hairdresser
or try on new clothes in a store and sometimes
the choice of career. Having to cover up
in warm weather and feeling unable to pursue
the activities of people who are unaffected
by the condition, add to the debilitating
features of living with psoriasis.
Temperature regulation is affected and
people with the condition can feel hot when
others feel cold, and vice versa. Sleep
can be difficult. Itching and soreness can
exacerbate the condition, leading to a general
feeling of being unwell.
It is important to
recognise that psoriasis is a non-contagious
condition and cannot be spread by touch
to unaffected areas of skin or to other
people.
Treatments
Mild to moderate psoriasis is treated with
topical applications containing coal tar,
dithranol, or the novel Vitamin A and D
derivatives. Ultra violet light therapy
and immunosuppressants are used in more
resistant cases.
For further
information contact:
Psoriasis Association, Milton House, 7 Milton
Street, Northampton, NN2 7JG.
Arthritis means inflammation
of a joint. Psoriatic arthritis is a form
of arthritis that occurs in people who also
suffer from psoriasis. In the UK, 2-3% of
the population develop some form of psoriasis.
10-20% of people with psoriasis develop
some form of psoriatic arthritis, with symptoms
usually starting between the ages of 30
and 50 (although the condition can occur
in teenagers too). In 80% of cases, the
arthritis appears after the skin symptoms.
In 20% of cases, the joint inflammation
comes first. There is no link between the
severity of the skin symptoms and the risk
of developing arthritis. Over 40% of people
with psoriatic arthritis have a family history
of the condition.
Symptoms
As with any type of arthritis, the inflamed
joints are tender, swollen and painful.
Joints also become stiff after resting,
especially after a night’s sleep. Other
symptoms include inflamed muscles and tendons,
especially around the elbows, wrists and
heels. Joints may lose their range of movement
and become deformed or locked.
Causes
A joint forms where two bones come into
close contact. Some joints are fixed (e.g.
in the skull) whilst in others the bones
can move more freely. The bone surfaces
in a mobile joint are protected from wear
and tear by slippery cartilage and a lubricating
fluid (the synovial fluid - produced by
the synovial membrane). Most joints are
held together by bands of tissue called
ligaments.
In psoriatic arthritis, the synovial membrane
becomes thickened and inflamed. It releases
more fluid than normal so that the joint
becomes tender and swollen. As inflammation
continues, it spreads to the cartilage underneath
and may eventually erode the bone. As the
tendons are lined and lubricated by synovial
membrane, these also become inflamed.
Types Asymmetrical oligoarticular - involves
one or more joints, especially the knees,
fingers and toes that may show sausage-like
swelling and redness.
Symmetrical rheumatoid-like
-involves the small joint of the fingers
or toes.
Distal interphalangeal
(DIP) - involves the last small joint
of the fingers or toes. Nail changes are
common.
Arthritis Mutilans
- a rare deforming condition of the joints.
Spondylitic -
inflammation of the spine and sacroiliac
joints.
Treatments
Aspirin-like drugs (non-steroidal anti-inflammatory
drugs) such as ibuprofen reduce the inflammation,
but can sometimes make skin symptoms worse
and can also cause indigestion or heartburn.
They should not be used in those with asthma.
Injections of a corticosteroid drug into,
or around, a single or painful joint can
relieve pain, increase mobility and reduce
deformity. Oral corticosteroid drugs are
best avoided. They can cause severe relapse
of the psoriasis in some people when they
are withdrawn. Disease modifying drugs switch
off the immune reactions that are causing
inflammation and pain. They do not have
any immediate effect and may take from 6
weeks to 6 months to work. It is important
to avoid pregnancy whilst taking these drugs
(effective contraception must be used during
treatment).
Psoriasis
(also see section on Psoriasis)
Psoriasis is a long-term (chronic) scaling
disease of the skin, caused by the over
production of skin cells. The skin shows
raised, red patches, which are often covered
with dead cells to form fine silvery scales
or thick white plaques. Pustules may form
when white blood cells (polymorphs) move
into the area. Psoriasis is a genetic disease
and hence there may be a family history
of the condition.
For further information
contact:
Psoriatic Arthropathy Alliance, PO Box 111,
St. Albans, Hertfordshire, AL2 3JQ.
The Psoriatic Arthropathy
Alliance is dedicated to raising awareness
and helping people with psoriatic arthritis
and its associated skin condition psoriasis.
Specifically, the PAA exists to:
promote self-help by encouraging good
patient compliance.
encourage early diagnosis.
campaign for better treatments and management
of the illness.
provide information to initiate appropriate
education projects.
work with medical and non-medical professionals
for the benefit of people with the condition.
encourage close working relations with
related UK and overseas organisations.
Raynaud's: Raynaud's
is a condition in which the blood supply
to the extremities, usually the fingers
and toes but sometimes also the ears and
nose, is interrupted. It is estimated to
affect 3-20% of the adult population and
is nine times more common in women.
Symptoms
During an attack the extremities become
first white and dead looking. They may then
turn blue and then red and burning. There
may be considerable pain, numbness or tingling.
The condition can range in severity from
minor discomfort, to the onset of ulcers
or even gangrene. Progress may be very slow
over a period of years and often starts
in the very young or early teens. Teenagers
are likely to grow out of it in their twenties.
Causes
These symptoms are due to an intermittent
lack of blood in the affected parts when
the arteries normally supplying them spasmodically
contract. An attack will often be triggered
by touching cold objects or exposure to
cold of any kind. It seems to be a change
of temperature rather than simple cold exposure
that triggers an attack, so it can occur
in the summer as well as in winter. Emotions,
such as anxiety, also play a part, as can
smoking. Raynaud's can occur spontaneously
or in association with other disorders such
as scleroderma, systemic lupus erythematosus
or rheumatoid arthritis.
People who work with
vibratory tools are prone to Raynaud's,
which appears to be permanent even when
the work with such tools has stopped. However
if identified early the symptoms may disappear
when the vibration ceases. This condition
is known as ‘vibration induced white finger’
and is recognised as an industrial disease
eligible for compensation.
Treatment
Treatment depends on the severity of the
condition and for those with a mild condition,
stopping smoking, wearing gloves and avoiding
the cold may be enough. If the condition
is severe, the General Practitioner can
advise on the different types of drugs available.
These include vasodilator drugs which open
up the small blood vessels.
Scleroderma The word scleroderma
means 'hard skin'. The condition affects
the blood vessels, immune system and connective
tissue. The skin, usually of the hands and
feet, becomes stiff, tight and shiny. The
disease is also known as systemic sclerosis,
when other parts of the body are affected.
It is estimated to affect 3,000 - 3,500
people in the UK.
Symptoms
In scleroderma the body produces too much
of a protein called collagen. This is essential
for holding the body together, but too much
causes the body to become stiff and unable
to function properly. This excess collagen
is like scar tissue, it causes thickening
and stiffening of those parts of the body
it affects. The disease may also affect
the connective tissue of the internal organs.
There are two major
types of scleroderma:
Localised scleroderma
- which affects isolated areas of skin
and the tissues beneath it. This is a relatively
mild condition and does not normally affect
internal organs.
Systemic scleroderma
- this affects both the skin and internal
organs, including joints, blood vessels,
the digestive system, heart, lungs and kidneys.
The symptoms include:
extreme sensitivity to cold (most people
with scleroderma suffer with Raynaud's at
some stage in their illness); swelling of
the hands and feet, especially in the morning:
and the skin of the fingers, toes, trunk
and limbs may become tight and shiny. The
joints may tighten and bend due to the thickening
of the skin. The disease usually starts
between the ages of 25 and 55, with women
being more often affected than men.
Causes
The cause is unknown but the condition is
neither contagious nor inherited.
Treatments
Scleroderma is a slow, chronic disorder,
usually starting slowly and progressing
gradually. Treatment consists of exercise,
care of the skin and various drugs to help
control the condition or treat complications.
Although there is no
cure, proper treatment and care can make
it possible for people with scleroderma
to lead a full life.
For further information
contact:
Raynaud's & Scleroderma Association, 112
Crewe Road, Alsager, Cheshire ST7 2JA. Please
enclose an A4 sae.
Rosacea is an inflammatory
condition mainly affecting the face. It
is hall-marked by episodic flushing of the
skin, often lasting for several hours. Some
people will periodically develop multiple
spots in the affected areas which often
become pustular. It is an under-diagnosed
condition which may affect up to 10% of
the population. There are two peak incidences
of rosacea, one in the 20s and one in the
50s, although people of any age may develop
the condition.
Causes
Rosacea can be hereditary and may run in
those with Celtic skin types. The flushing
attacks may be triggered by a number of
different factors. Often these trigger factors
will vary considerably from individual to
individual. No exclusion list exists that
is suitable for everyone. There are, however,
many common triggers including:
hot (temperature)
dairy products food and drinks
alcohol
caffeine
spicy foods
stress
temperature changes
sun
wind
embarrassment
Symptoms
After a period of intermittent flushing,
the skin becomes persistently red with the
development of multiple telangiectasia (tiny
broken veins) over the affected area. Experiencing
these flushing attacks can be extremely
uncomfortable. Attacks can vary from burning
to intense pain. Rosacea also causes considerable
embarrassment itself. For example, 23% of
members of the Acne Support Group with rosacea,
who have taken part in public surveys, said
that they have been asked whether they had
a drinking problem.
Treatments
Many people believe their facial redness
is purely a cosmetic problem and do not
seek advice about it. However, it is important
to treat this condition early to prevent
long term side effects which include: telangiectasia;
rhinophyma (enlargement of the nose); persistent
oedema of the face (swelling) or eye problems.
Treatment should start with identifying
possible trigger factors. It could be helpful
to keep a diary over a few weeks to help
to identify foods which might make the flushing
worse. In mild rosacea, topical antibiotics
are commonly used. In general, metronidazole
is used and needs to be applied sparingly
to the whole affected area. In those who
fail to respond to topical antibiotics,
or those with a more severe disease, systemic
antibiotics can be used. Systemic antibiotics
tend to have their major impact on the inflammatory
lesions, with the flushing tending to respond
less quickly. Roaccutane, a hospital-only
drug, can also be prescribed to some people,
but there are many reported side effects
of this treatment, so it should be considered
carefully before using. If flushing is a
major problem, Clonidine can help to reduce
flushing attacks.
Rosacea can be confused
with acne or seborrhoeic dermatitis, although
some people have both rosacea and seborrhoeic
dermatitis. Therefore it is important that
the condition is diagnosed correctly, so
that the most appropriate treatment is administered.
It is considered that
rosacea is often self-limiting, but it is
impossible to predict how long it may last.
Many people affected by this skin condition
can feel embarrassed and ashamed by their
appearance. There are some excellent camouflage
creams available from the British Red Cross
Skin Camouflage Service, which can help
to tone down facial redness and are suitable
for both men and women.
The key to managing
rosacea is to be the one in control of the
skin condition, and not the other way around.
For further information
contact:
Acne Support Group, PO Box 9, Newquay TR9
6WG.
The Acne Support Group
provides information and support to those
people affected by acne and those people
affected by rosacea. Information and services
available to members include:
Varicella-zoster
virus is the virus that causes shingles
and chickenpox. Chickenpox is almost universal
in the UK with 95% infection rate by age
21. However in other parts of the world,
childhood chickenpox is less common leaving
immigrants from developing countries susceptible
to infection in adulthood with potentially
serious consequences.
This virus, like the
other eight human herpes viruses, remains
latent in the body and may reactivate in
response to ill-health, stress or trauma
to the area. Shingles is the name given
to these recurrences and is more likely
to affect older people (50% at 85 years).
Post herpetic neuralgia (PHN) follows shingles
in a rising proportion of older patients:
from 50% of 60 year olds with shingles to
70% of those aged 75.
Causes and Symptoms
Chickenpox (herpes varicella) is very infectious.
It is self-limiting and rarely serious in
children. Adults may, however, become seriously
ill. After an incubation period of 14-21
days a febrile illness will develop followed
by typical spots over most of the body.
These may develop into ulcers before scabbing
over and healing.
After the primary infection,
herpes viruses remain latent in the body
and may reactivate at a later date.
Varicella-zoster virus
is called shingles if it recurs and the
vesicles (spots) are localised, usually
on head or chest. Shingles (herpes zoster)
cannot be caught although it may be passed
to susceptible persons as chickenpox.
In
shingles, the vesicles and typical pain
follow the line of the nerve. Neurological
damage can leave symptoms, described variously
as excruciating pain, scalded skin, deep
aches, tingling, intolerable itching or
shooting nerve pains. This post herpetic
neuralgia may last months or years.
Treatments
If the primary infection of chickenpox is
severe, and when a shingles occurs in an
older patient, one of three antiviral tablets
may be prescribed: aciclovir, Famvir or
Valtrex. Otherwise all that is necessary
is to alleviate symptoms: antipyretics,
analgesics, topical anaesthetics as appropriate.
Eye infection:
Varicella-zoster virus may affect the ophthalmic
division of the trigeminal nerve causing
pain in, usually, one eye. Diagnosis is
by fluoroscein stain. Treatment with antiviral
ophthalmic cream as well as antiviral pills
is recommended. Eye infection may damage
sight or even, rarely, lead to blindness.
Because postherpetic
neuralgia (PHN) becomes more prevalent with
age, over 50s should be started on prescribed
treatments to block PHN: a low-dose tricyclic
anti-depressant.
If PHN develops gabapentin
may be added to the drug regimen. Capsaicin
cream has been successfully used in several
trials.
Self-help such as firm
bandaging or use of a TENS (transcutaneous
electrical nerve stimulation) unit may alleviate
the pain.
Reported
PHN should not be dismissed by doctors as
the constant sensations ranging from constant
itching to acute pain may render the patient's
final years an unendurable misery. Suicides
have been recorded.
For further information
contact:
Shingles Support Society c/o Herpes Viruses
Association (SPHERE) 41 North Road London
N7 9DF Reg. Charity No. 291657
Tel: 020 7607
9661 (office and Minicom V) Fax: on request
The
Shingles Support Society is a sub-group
of the Herpes Viruses Association (see “herpes
simplex”). To receive a 19 page information
pack on treating post herpetic neuralgia
please send sae (and small donation). The
pack includes:
Introductory sheets on chickenpox and
shingles (2 pages)
Tips from other sufferers on dealing
with PHN (2 pages)
Drug treatments and dosage sheets, fully
referenced, for GPs – written by Dr David
Bowsher, consultant neurologist, of the
Pain Relief Foundation (7 pages)
Leaflet on TENS (transcutaneous electric
nerve stimulation) (2 pages)
Leaflet on dealing with chronic pain
(2 pages)
A list of names and addresses of people
with shingles who would like to communicate
with others (2 pages)
Skin cancer is the
second most common cancer in the UK with
over 40,500 new cases every year. There
are three main types of skin cancer: basal
cell carcinoma (BCC), squamous cell carcinoma
(SCC) and malignant melanoma (MM). Due to
certain similarities basal cell and squamous
cell carcinoma are often grouped together
and referred to as non-melanoma skin cancer.
The prevalence of BCC in the population
is 2.1%. BCC is twice as common as SCC.
MMs account for 1-in-10 skin cancers.
Types and Causes Basal cell carcinoma arises from
the cells in the base of the skin and is
the most common skin tumour in the UK. It
is usually seen in caucasians, particularly
those with fair complexion, fair hair and
blue eyes. The type of skin affected is
almost always hair bearing skin, though
occasionally basal cell carcinoma is found
on the soles of the feet. Most basal cell
carcinomas are slow growing and do not spread.
However, if left, they can erode the skin
and cause an ulcer, known as a rodent ulcer.
Squamous cell carcinoma starts in
the surface cells of the skin and is the
second most common type of skin cancer in
the UK. This is a slow growing cancer but
may spread to other parts of the body if
left untreated. Like basal cell carcinoma,
squamous cell carcinoma tends to occur in
caucasians or white skinned people, with
more males than females being affected.
Malignant melanoma,
although not the most common form of skin
cancer, does cause the greatest concern
as it is curable if found early, but can
be very difficult to cure if it has spread
into the deeper layers of the skin. MM develops
in cells known as melanocytes, which are
responsible for the colour of our skin.
This type of cancer usually starts in the
skin. Rarely it can arise in other parts
of the body, such as the eye, the mouth,
or in the internal organs.
There is strong evidence
that ultraviolet (UV) rays from the sun
or sun beds can damage the skin and cause
cancer. The likelihood of developing a skin
cancer increases with age and non-melanoma
skin cancer is more common in those over
40 years old.
Certain risk factors
have been identified. These include people
who are fair skinned, burn easily in the
sun, are red-haired with freckles, have
sunspots (solar keratoses), have had previous
skin cancer and those who work or spend
long periods outdoors. The risk of developing
skin cancer can be reduced by cutting down
on exposure to ultraviolet light and increasing
protection measures. These include wearing
protective clothing, wide brimmed hats and
using a strong sun block (minimum SPF 15).
Children and young adults who are over-exposed
to the sun and suffer blistering or burning
are at increased risk of developing a MM
in later life. Very rarely, malignant melanoma
may be due to a genetic or familial tendency
to the disease.
Symptoms
Both basal and squamous cell skin cancers
can appear anywhere on the body but are
more likely to appear on exposed skin, especially
the face, neck, arms, hands and lower legs.
They can appear in a variety of forms: a
small lump on the skin (which is smooth
and pearly or waxy in appearance, or which
bleeds, crusts and does not heal, or is
wart-like in appearance); a flat red spot
or a firm red lump.
Most MMs start in normal
skin. Others may develop in existing moles.
If it does develop from a mole, changes
include: a change in size; altered shape;
change in colour; itching, crusting or bleeding.
People should be encouraged to report any
unusual marks on the skin which last more
than a few weeks or an existing mole which
shows any of the above signs.
Many people feel overwhelmed
when told they have a diagnosis of skin
cancer. The impact can be particularly devastating
if the cancer is on a prominent part of
the body, such as the face, neck or arms/hands.
The prospect of potentially disfiguring
treatment can be hard to cope with. People
in this situation need accurate information
and sensitive support to help them understand
what is happening.
Treatments Treatment options include: surgery;
electrocautery; cryosurgery; lymph gland
removal; radiotherapy; chemotherapy and,
immunotherapy (for MM). These treatments
may be used alone or in combination. Most
people with non-melanoma skin cancer are
cured, whilst the prognosis for MM depends
on the depth of the cancer in the skin.
It is important to report a MM early.
For further information
contact:
Cancer Research UK PO Box 123 London WC2A
3PX Tel: 020 7242 0200 www.cancerresearchuk.org
Cancer Research UK
is dedicated to research on the causes,
treatment and prevention of cancer.
Wessex Cancer Trust’s
Marc’s Line, Marc’s Line Resource Centre,
Dermatology Treatment Centre, Level 3, Salisbury
District Hospital, Salisbury, SP2 8BJ. Tel:
01722 415071. Website: http://www.wessexcancer.org/
(part of the Wessex Cancer Trust Cancer
Information Network)
T uberous sclerosis
is an autosomal dominant disorder of variable
expression, characterised by extra tissue
growths, hamartomas, in almost any organ
of the body. Hamartomas include cortical
tubers and subependymal nodules in the brain,
retinal phakomas, renal cysts and angiomyolipomas,
cardiac rhabdomyomas, pulmonary cysts and
a variety of cutaneous stigmata - hypomelanic
macules, facial angiofibroma, ungual fibroma,
shagreen patches and forehead plaques. It
is associated with learning disability (in
up to 50% of cases), epilepsy (60-70%) and
autistic traits (50%). Up to 90% will have
some skin signs, and in some cases these
will be the only clinical feature of the
condition. The condition affects between
1-in-8,500 to 1-in-10,000 people.
Causes Tuberous sclerosis is caused by a defect
in cell growth regulating genes, either
on chromosome 9 or on chromosome 16. Although
approximately two thirds of cases are sporadic,
it is essential that genetic counselling
is offered to families after diagnosis because
of the variability of the disorder. A diagnosis
is presently confirmed by clinical and radiological
investigations. DNA tests, including pre-natal
testing, are now possible for some families.
Symptoms Tuberous sclerosis can present at any
age, with symptoms ranging from seizures
or cardiac arrhythmias in infancy, to the
later appearance of facial angiofibromas
or renal angiomyolipomas. Ensuing investigations
may subsequently reveal hypomelanic macules
(90%), which fluoresce under ultra-violet
light, calcified subependymal nodules (80%),
which show up on a CT brain scan, and, in
neonates, cardiac rhabdomyomas (60%). Life
expectancy is generally good, even for those
with severe learning disability. Infrequently,
death can occur from brain tumours or kidney
disease, but most people have good long-term
prospects if monitored appropriately for
signs of serious problems.
Skin signs which present
from birth onwards are hypomelanic macules
(90%), which can repigment in adult life,
and smooth, fibrous forehead plaques (25%).
Shagreen patches (40%), thickened discoloured
skin or nodular lesions, are more likely
to develop later in the lower lumbar area.
Facial angiofibromas (85%) can appear at
any age from two years upwards, starting
as tiny red dots and developing during adolescence
across the nose, upper cheeks, and particularly
in the chin and nasolabial folds. This rash
used to be misleadingly called adenoma sebaceum.
Ungual fibromas (50%)
can grow from the nails of both hands and
feet, from late adolescence onwards causing
grooves in the nails even if the lesion
cannot be seen. Skin tags occur in 30% of
people affected by the condition, and can
be profuse at the back of the neck, across
the shoulders, in the groin or on the testes.
Treatments Since there is no cure for tuberous
sclerosis, treatment has to be symptomatic.
Epilepsy is treated with drugs (or occasionally
surgery); raised intracranial pressure can
usually be treated by surgical removal of
the tumour and/or the insertion of a shunt;
renal problems may require embolisation
of an angiomyolipoma or more rarely partial
nephrectomy, dialysis or transplantation;
symptomatic lung disease may respond to
treatment with progesterone; and cardiac
problems only rarely require medical treatment.
Behavioural difficulties (including hyperactivity,
autistic features, sleeping problems and
hallucinations) are often the most troublesome
for families and frequently require appropriate
intervention.
Of the various skin
manifestations associated with tuberous
sclerosis, there are three which may benefit
from treatment. The forehead fibrous plaques
and similar growths on the head can sometimes
enlarge considerably in late adolescence
and require plastic surgery. The ungual
fibromas can be removed either by laser
or diathermy (although they usually regrow
whatever treatment is used). However it
is the facial angiofibroma which cause the
most psychological distress to patients
and these can also bleed profusely if rubbed.
These angiofibromas benefit most from the
laser treatments, which are currently thought
to be the most effective and least traumatic
treatments. The argon and pulsed dye lasers
have more success on the vascular lesions,
whilst the carbon dioxide laser is more
successful where there is a greater fibrous
component to the lesions.
For further information
contact: Tuberous Sclerosis Association, PO
Box 9644, Bromsgrove, Worcestershire B61
0FP.
Vitiligo
is one of the most common skin diseases,
and yet the vast majority of people have
never even heard the name. Probably because
vitiligo is not life threatening, statistics
on its prevalence are not routinely collected.
On the basis of various clinical studies
around the world, it is generally estimated
to affect 1-4% of all races and populations.
This implies that over half a million people
in the UK alone have vitiligo.
Symptoms
and Causes
Vitiligo can affect anyone of any age and
either sex. In those who have the condition,
patches of skin turn white, and in some
cases their hair can also lose its colour
in patches. Although it has no effect on
the patient’s general physical health, the
white patches usually increase over time
, producing further patches with patches
joining together to form large areas of
totally white skin and are highly vulnerable
to sunburn, even when only briefly exposed
to mild sunshine. On exposed areas such
as the face and hands it can be very disfiguring,
especially on dark or tanned skin. Many
people who have vitiligo find it socially
and psychologically devastating, and can
lose their self esteem and self confidence.
The
causes are not yet properly understood,
but there is growing evidence which indicates
that the disease is genetic in origin in
most cases.
There
are other skin conditions, particularly
fungal infections, which can easily be mistaken
for vitiligo. If there is any doubt about
the diagnosis, a referral to a hospital
dermatology (skin) clinic is advised.
Treatments
An
outright cure has not yet been found, but
there are recognised National Health Service
(NHS) treatments which can sometimes bring
colour back into the white patches or at
least control their spread, although there
is no guarantee that they will work in any
individual case.
One
of these treatments is the use of steroid
creams on the affected areas at the onset
of the disease. This can sometimes stop
the spread of the patches or, even restore
some of the lost colour. This treatment
is usually given only for a limited period
under medical supervision.
Another
NHS treatment involves the use of ultraviolet
light. One of these treatments, known as
PUVA (psoralen + UVA), can encourage the
natural colour to come back within the white
patches. This treatment involves the use
of drugs called psoralens, which make the
skin sensitive to light. The skin is then
exposed to ultra-violet A light . In the
UK, PUVA is normally available only as a
hospital outpatient treatment, and it should
always be supervised by a dermatologist
(skin specialist).
A
more recent development is the use of narrow-band
ultraviolet B light This has the advantage
that no drugs need to be taken, and the
repigmentation is often more successful
with this method than with PUVA. In the
UK this treatment is usually only available
in hospitals under the supervision of a
dermatologist.
A
further development in research, has shown
that psychological counselling using cognitive
behavioural therapy can both help the patient
to recover his/her self-esteem and can,
in some cases, help lost colour to return
to the white patches.
Complementary
treatments, including herbal and homoeopathic
remedies, can be very expensive. They may
sometimes work, but there is no proof of
this and there could be unforeseen side
effects. The patient should be cautious
and should never take any medicine or pills
without knowing what they contain. It is
important to inform the General Practitioner
about any treatments undertaken, and to
make sure that the complementary practitioner
consulted is properly qualified and registered
with a recognised professional body. Anyone
who claims to offer a ‘cure’ should be avoided.
For
those who are troubled by their appearance,
concealing the white patches may improve
self confidence. There are cover creams
which are specially designed for this purpose
and are suitable for use by men, women and
children alike. Some of these, classified
by the NHS as ‘borderline substances’, can
be prescribed by doctors. The Skin Camouflage
Service of the British Red Cross has a nationwide
network of clinics which advise on the use
of cover creams. Some people prefer to use
self-tanning creams (sometimes called fake
tans), which are available over the counter
at most chemists or beauty counters.
It
is important to protect the white patches
from burning in the sun. These patches are
wholly or partly lacking in the pigment
which is the skin’s natural protection against
sunburn. Allowing the skin to burn can encourage
the vitiligo to spread. The patches should
be protected from the sun by clothing, or
by the use of a high factor sunblock. Some
brands of sunblock, which have a sun protection
factor of 25 or above are classified as
borderline substances and can be obtained
on prescription.
Children
and young people
Children
with vitiligo cope best if parents answer
their questions about their condition truthfully
and simply. They need to be reassured that
it will not affect their family’s feelings
towards them. They can have problems with
bullying and teasing at school, which may
be overcome by explaining vitiligo to teachers
and enlisting their help.
For
further information contact: Vitiligo Society, 125 Kennington Road,
London, SE11 6SF.
Xeroderma Pigmentosum (XP) is a rare genetic
disorder that may cause extreme sensitivity
to the sun's ultraviolet rays. Unless patients
with XP are protected from sunlight, their
skin and eyes may be severely damaged. This
damage may lead to cancers of the skin and
eye. XP has been identified in people of
every genetic group all over the world.
There are about 100 confirmed cases in the
UK.
Causes Ultraviolet light damages the DNA
in cells and disrupts normal cell functioning.
DNA (deoxyribonucleic acid) within our genes
contains all the coded information needed
to direct cell functions.
Two factors combine to cause the abnormalities
in XP. Firstly, a person inherits traits
from each parent which, when combined, lead
to an unusual sensitivity to damaging effects
of ultraviolet light. Secondly, exposure
to the sun, which contains ultraviolet light,
leads to changes in the skin and eyes.
Damaged DNA is repaired by the DNA repair
system. But the DNA repair systems of people
with XP do not function properly. As a result,
un-repaired DNA damage builds and causes
cancerous cell changes or cell death.
Symptoms Many people with XP get unusually severe
sunburn after a short period of sun exposure.
The sunburn may last much longer than expected,
perhaps for several weeks. This type of
sunburn will usually occur during a child's
first exposure, and it may be a clue to
the diagnosis of XP. However, some people
with XP do not burn more easily and the
disease will be undetected until unusual
skin changes appear over time.
Most patients with XP develop freckles
at an early age. Continued sun exposure
will lead to further changes in the skin,
including irregular dark spots, thin skin,
excessive dryness, rough-surfaced growths
and skin cancers. These skin changes will
resemble those of elderly people who have
spent many years in the sun. In people with
XP, the changes caused by sun damage often
begin in infancy and almost always before
the age of 20.
The eyes of a person with XP are often
painfully sensitive to the sun and may easily
become irritated, bloodshot and clouded.
Non-cancerous and cancerous growths on the
eyes may occur.
Treatments
There is no cure for XP, but much can be
done to prevent and treat some of the problems
it causes:
Protection from ultraviolet light, by
a combination of physical and chemical
means. These include sun avoidance, shade,
clothing (including hats), optical filtration
and sunscreens.
Frequent skin and eye examinations.
Prompt removal of cancerous tissue.
Neurological examination in some cases.
Text extracted from: 'Understanding Xeroderma
Pigmentosum' published by US Department
of Health & Human Services (by permission).
For further information contact: XP Support Group, 2 Strawberry Close,
Prestwood, Great Missenden, Bucks., HP16
0SG. Reg. Charity No. 1075302.
The XP Support Group is a charitable trust
set up to continue the work of the Xeroderma
Pigmentosum Society Inc., a non-profit organisation
based in New York State, USA. Parents of
children with XP set up both the XP Society
(USA) and the XP Support Group (UK). The
XP Support Group is independent of the XP
Society.
The aims of the Support Group are:
to relieve the needs of people with
Xeroderma Pigmentosum and UV-related conditions
and their families;
to advance the education of the public
in Xeroderma Pigmentosum;
to promote research into Xeroderma Pigmentosum.
To achieve these aims the Group will
endeavour to:
raise funds for the XP Research fund
set up by the XP Society;
assist families to attend Camp Sundown
(a night-time camp held once a year in
the USA) or respite in a protected environment.
Our respite home is at St Katharine’s,
Frieth in Buckinghamshire, and is where
we hold our annual night-time camp called
the “Owl Patrol”;
give grants for UV protective products;
raise public awareness by means of an
education campaign.
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