Hailey-hailey disease

The disease is caused by a change in the DNA of a gene on chromosome 3. Normally this gene helps the cells in the outer layer of the skin (the epidermis) to 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. This happens especially in areas where the skin is rubbed 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 and is not 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. The severity may vary between affected members of the same family. While it is hereditary not all patients have affected family members.

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-ups tend to settle down in a few days with appropriate treatment although sometimes they may improve without treatment.

Severe and painful flare-ups in an area such as the groin, genital area or in the crease of the buttocks may make walking and working so uncomfortable that an individual may need a few days of rest. Severe flare-ups may lead to infection and discharge, which may cause increased pain or a strong smell from the affected areas. The affected areas may become particularly problematic if secondarily infected. This may be due to bacterial and/or viral (particularly the cold sore virus) infection.

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 and in the skin folds (under the arms, groin or under the breasts). The skin looks normal in between flare ups, and does not scar. However some patients may find that there is a brown discolouration that can occur as a result of the inflammation (post-inflammatory hyperpigmentation), which can take months to fade. Some people notice that their condition improves as they get older.

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 taking a sample of the skin (a biopsy) for examination under the microscope. A second biopsy is often taken for direct immunofluorescence to confirm the diagnosis.

Can Hailey-Hailey disease be cured?

No. The underlying genetic defect cannot be altered; however, treatment does help with prevention of flare-ups and may increase the speed of healing.

How can Hailey-Hailey disease be treated?

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

Heat, sweating or friction may make the rash develop; it is sensible to try and avoid these where possible. 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 loose fitting clothing, and keep your weight down to minimise friction. Avoid sweating and friction by doing exercises such as swimming, rather than activities such as cycling.  Holidays in hot and humid countries may make the condition worse.