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Patient Information Leaflet

Hailey-Hailey Disease

What are the aims of this leaflet?

This leaflet has been written to help you understand more about Hailey-Hailey disease. It tells you what it is, what causes it, what can be done about it, and where you can find out more about it.

What is Hailey-Hailey disease?

Hailey-Hailey disease is also known as familial benign chronic pemphigus. It is a rare inherited skin condition, in which red scaly areas or small blisters appear at sites of friction. People usually lead full and normal lives, with their condition being a nuisance rather than a serious problem.

What causes Hailey-Hailey disease?

A small error in the DNA code for a gene on chromosome number 3 (one of the 46 chromosomes that we all have) has been shown to cause Hailey-Hailey disease. Normally this gene plays a part in ensuring that the cells in the outer layer of the skin (the epidermis) stick together properly. In Hailey-Hailey disease, this adhesion of cells is impaired, resulting in separation of the cells in the epidermis, especially in areas of skin prone to friction such as the groin and under the arms. The altered gene runs in families and affects both men and women. Hailey-Hailey disease is not contagious, or a result of allergies. There is a tendency for Hailey-Hailey disease to get worse with sweating and friction, as well as in hot weather.

Is Hailey-Hailey disease hereditary?

Yes – see above. The condition is inherited in a pattern known as 'dominant inheritance', which means that there is a 1 in 2 [50:50] chance that each child of an affected parent will inherit the skin problem.

What are the symptoms of Hailey-Hailey disease?

Affected skin may become uncomfortable and itchy if the skin surfaces are continually rubbing against each other, such as in the groin when walking. Outbreaks tend to settle down in a few days with appropriate treatment (see below), and may improve without treatment. Very rarely, a severe flare-up in an area such as the groin, genital area or in the crease of the buttocks may make walking and working so uncomfortable that a patient might need a few days rest.

What does Hailey-Hailey disease look like?

The first signs of the condition usually appear between the ages of 15 and 40 years, but it may begin outside this age range. The severity varies enormously and is unpredictable. Red, scaly areas or small blisters appear at areas of friction, especially at the sides of the neck or in the skin folds under the arms, the groin or under the breasts. The skin looks entirely normal inbetween active episodes, and does not scar. Many patients notice that their condition improves as they get older.

How will Hailey-Hailey disease be diagnosed?

It is very common for the condition to be mistaken for eczema, contact dermatitis (due to an allergy), a sweat rash, a fungal infection or impetigo (a bacterial skin infection). If required, the diagnosis of Hailey-Hailey disease can be confirmed by examining a sample of the skin (a biopsy) under the microscope.

Can Hailey-Hailey disease be cured?

No. The underlying genetic defect cannot be altered; however, treatment does help and long remissions are common.

How can Hailey-Hailey disease be treated?

There are several ways of helping people with Hailey-Hailey disease:

• Corticosteroid creams are very helpful and may be prescribed in a mix with an antibiotic to prevent skin infection. A pricking or itching sensation is usually present when a spot is starting and the corticosteroid cream may prevent the spot from developing any further if the cream is used as soon as you are aware of the itching sensation. The cream should be applied very thinly, and only needs to be used once or twice a day on the affected skin. There is no need to put any cream on unaffected skin. 
• The affected skin may smell unpleasant, particularly in moist areas. The smell is part of the skin condition and does not mean that the skin is dirty; bacteria or yeasts, growing in the rash, probably cause it. Careful washing is important and antiseptic solutions for the bath, antiseptic creams, antibacterial soaps, anti-yeast creams or oral antibiotics may help. 
• There does seem to be an increased chance of developing a skin infection if you have Hailey-Hailey disease. Infection with the same sort of bacteria that infect cuts or grazes is common and may make your condition worse – this may require treatment with antibiotics from your doctor. The "cold sore" virus (herpes simplex) sometimes causes widespread skin infections in people with Hailey-Hailey disease. If your condition suddenly gets worse and is much more painful than usual this may be a sign of a cold sore virus infection. You should contact your GP or dermatologist immediately if this happens, as you may need anti-viral treatment. 
• More recently topical tacrolimus cream has been found useful in a number of patients, although this preparation does not have a product licence to treat Hailey-Hailey disease. 
• Occasionally treatments which affect the immune system may be used (e.g. corticosteroids, ciclosporin, dapsone or methotrexate); these all, however, have potentially serious side effects so are only used in patients who do not respond to less severe treatment.
• Topical photodynamic therapy, a type of light treatment, has been reported to help in some but not in other resistant cases. This involves the application of a cream that sensitises the cells to light, followed by exposure to a special red lamp 3 hours later. The treatment can be painful. 
• Other possible approaches to treatment that are still being assessed include the use of botulinum toxin (to reduce sweating), the removal of affected areas surgically (which may result in scar formation), and laser treatment.

Self care (What can I do?)

Heat, sweating or friction may make the spots appear and it is sensible to try and avoid these; for example, it may be helpful to wash a new shirt to soften a stiff collar before wearing it. It is advisable to wear cool comfortable clothing, and keep your weight down to minimise friction. Avoiding sweating by doing exercises such as swimming, rather than activities where you sweat a lot, is worth considering; holidays in hot and humid countries may make the condition worse.

Where can I get more information about Hailey-Hailey disease?

Links to patient support groups:

Hailey-Hailey Disease Society (U.S.A)
www.haileyhailey.com
E-mail: info@haileyhailey.com

Web links to detailed leaflets:

http://emedicine.medscape.com/article/1063224-overview (include photographs)
www.dermnetnz.org/dna.Hailey-Hailey/HHD.html (include photographs)

The British Skin Foundation fund vital research into all skin diseases. To find out how you can help, please visit the British Skin Foundation website here.

This leaflet aims to provide accurate information about the subject and is a consensus of the views held by representatives of the British Association of Dermatologists: its contents, however, may occasionally differ from the advice given to you by your doctor. 

This leaflet has been assessed for readability by the British Association of Dermatologists’ Patient Information Lay Review Panel

BRITISH ASSOCIATION OF DERMATOLOGISTS
PATIENT INFORMATION LEAFLET
PRODUCED FEBRUARY 2008
UPDATED JANUARY 2011