Dermalogica ®Store
Frequently Asked Questions

dermalogica: skin care and skin treatment dermalogica skin care and skin treatment creams, cleansers, lotions

skin care and dermalogica skin treatment products, skin treatment creams, cleansers, lotions

Page Menu

Please wait until the page is loaded, then choose a category from the above list, this will take you to the correct section of the page.

ACNE



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.

Tel: 0870 870 2263
Email: alison.dudley@btopenworld.com
Website: www.stopspots.org

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:

  • a comprehensive information pack.
  • confidential advice.
  • a lively and informative newsletter.

Return to the Top of Page



ALOPECIA

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.
  • campaigns to raise public awareness.

Return to the Top of Page


BULLOUS PEMPHIGOID

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.

Return to the Top of Page



DARIER'S DISEASE

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.

Website: http://www.dariers.8m.com

The Darier's Disease Support Group provides information and support to people with Darier's disease, with the motto:

  • Do not give up hope.
  • Awareness to others.
  • Readiness to smile.
  • ADefinite goal.
  • Isolation no longer.
  • Speak and be heard.

Return to the Top of Page



ECTODERMAL DYSPLASIA

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

Website: www.ectodermaldysplasia.org Contact: Mrs. Diana Perry

The Ectodermal Dysplasia Society aims to:

  • 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.

Return to the Top of Page



ECZEMA

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

Eczema Information Line: 0870 241 3604, email: helpline@eczema.org

Websites: www.eczema.org

Professional members' site: www.eczemapro.org

The National Eczema Society is dedicated to meeting the needs of people with eczema and their families by:

  • providing support and information on the disease and its management
  • producing publications on eczema and its management
  • providing information for health care professionals and a professional membership scheme
  • funding research into causes of and treatments for eczema

Return to the Top of Page



EHLERS-DANLOS SYNDROME

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.

Reg. Charity No. 1014641. Tel/Fax: 01252 690940. E-mail: director@ehlers-danlos.org

Website: http://www.ehlers-danlos.org/

Return to the Top of Page



EPIDERMOLYSIS BULLOSA

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.
  • Junctional epidermolysis bullosa -with pyloric atresia.

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.

Reg. Charity No. 1084958.

Tel: 01344 771961. Fax: 01344 762661.

Email: debra.uk@btinternet.com

Website: http://www.debra.org.uk


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.

Return to the Top of Page



HERPES VIRUSES

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

Reg. Charity No. 291657

Tel: 020 7607 9661 (office and Minicom V)
Tel: 020 7609 9061 (helpline – 24 hours access)
Fax: on request Website: http://www.herpes.org.uk

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.

Return to the Top of Page



HIDRADENITIS SUPPURATIVA

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.

Website: http://www.hssg.org.uk

Support Community website: http://www.communityzero.com/ ukhsgroup/

The Group aims to:

  • provide support for people with hidradenitis suppurativa and their families.
  • promote higher awareness of hidradenitis suppurativa within the UK and worldwide.
  • educate doctors about the effects of the condition.
  • have hidradenitis suppurativa recognized as a disability by the UK Government.
  • establish funding for research into treatments and to find a cure for hidradenitis suppurativa.

Return to the Top of Page



HYPOMELANOSIS OF ITO

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