Hailey-Hailey disease

What is Hailey-Hailey disease?

Hailey-Hailey disease is also known as familial benign chronic pemphigus, as originally described by the Hailey brothers. It is a rare inherited skin condition in which red scaly areas that can be itchy and sore, can lead to superficial blisters and eroded (broken) areas of the skin folds of the groin, armpits, neck and under the breasts. The condition flares intermittently and tends to come and go. Many patients are able to lead full and normal lives, with their condition being a nuisance rather than a serious problem. Some patients are more severely affected and experience more persistent painful raw areas of the skin with development of superficial blisters. 


What causes Hailey-Hailey disease?

A small error in the DNA code of 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. In Hailey-Hailey disease this adhesion of cells is weakened, resulting in separation of the cells in the surface layer of the skin, 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 equally. 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. The condition is inherited in a pattern known as 'autosomal 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?

During a flare-up, the affected skin may become uncomfortable, with burning and itching symptoms. This is aggravated if the skin surfaces are continually rubbing against each other, such as in the groin when walking. Flare-up tend to settle down in a few days with appropriate treatment although sometimes it may improve without treatment.

Severe and painful 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 of rest. Severe flare-ups may be lead to infection and discharge and may lead to an unpleasant smell from the affected areas.

What does Hailey-Hailey disease look like?

The condition usually first appears between the ages of 15 and 40 years, but it may begin at any age. The severity of the condition varies widely and is unpredictable. Red, scaly areas, superficial blisters or raw areas appear at areas of friction, especially at the sides of the neck, in the skin folds under the arms, groin or under the breasts. The skin looks normal in between flare ups, and does not scar. Some 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 other skin conditions such as eczema, psoriasis, contact dermatitis (due to an allergy), fungal infection or impetigo (a bacterial skin infection). The diagnosis of Hailey-Hailey disease can be confirmed by examining a sample of the skin (a biopsy) under the microscope. A test called direct immunofluorescence is also performed on a skin biopsy to confirm the diagnosis.

Can Hailey-Hailey disease be cured?

No. The underlying genetic defect cannot be altered; however, treatment does help with varying periods of remission.

How can Hailey-Hailey disease be treated? 

To find out about available treatments please visit this page on the website of the British Association of Dermatologists. 


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