Hailey-Hailey disease

Hailey-Hailey disease is a rare disease of the skin. It can begin at any age, but it is most common in the late teenage years, twenties and thirties. Typically it appears as thin walled blisters that easily rupture into erosions. The blisters are rarely seen because they break so easily. Hailey-Hailey disease can occur anywhere on the skin, but it is more frequently seen in the body folds such as the groin and armpits and neck.

A tiny fault in the DNA code for a gene on chromosome number 3 has been shown to reason Hailey-Hailey disease. In general this gene plays a part in ensuring that the cells in the outer layer of the skin (the epidermis) stick together properly, for example when they are rubbed. The altered gene runs in certain families and affects both men and women. Hailey-Hailey disease is not contagious or due to allergies. It tends to get worse with sweating and friction, and in hot weather.

People with Hailey-Hailey disease generally lead full, normal lives their condition being a nuisance rather than a grave problem. The lesions are most normally seen in the sides of the neck, armpit, and groin areas and under the breast. These patients may complain of a heavy smell and itching, which may be a social distress to patients’ lives. Unfortunately there is no cure for Hailey-Hailey disease. Treatment is aimed at reducing symptoms and preventing flares.

There are several ways of helping people with Hailey-Hailey disease. The first part of treatment is to avoid friction and sweating by wearing lightweight clothes. Avoiding direct sun and using sunscreen may also be helpful. Combination corticosteroid/ antibiotic creams are helpful, as treating the secondary infection is important. Topical photodynamic therapy may help in particularly resistant cases. In severe cases surgery can be performed to remove the affected skin.