HAILEY-HAILEY DISEASE (Benign Familial Chronic Pemphigus)

This patient information and photograph on Hailey-Hailey Disease is provided by John L. Meisenheimer, M.D. a board certified Dermatologist and skin care specialist based in Orlando, Florida. This information is not intended as a substitute for the medical advice or treatment of a dermatologist or other physician.

What is it?

Hailey-Hailey disease is a rare disease of the skin. It can begin at any age, but it is more commonly seen in adults. 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.

What causes it?

Those with benign familial chronic pemphigus have a decreased number of desmosomes in the skin. Desmosomes are like little rivets that hold the skin cells together. Because the skin cells are not as attached together as well as normal skin, conditions such as heat and frictional pressures may cause Hailey-Hailey disease to flare.

Benign Familial Chronic Pemphigus is a genetic disease. The predisposition to get this disease is carried within your genes. Children of affected people have a fifty-fifty chance of also getting the disease. About one third may get the condition without a family history.

Is it dangerous?

For the typical healthy person Hailey-Hailey disease is a harmless disorder, but it can be frustrating. It is not related to cancer and it does not involve internal organs.

Can it be cured?

The treatment program I put you on will help control or clear the Benign Familial Chronic Pemphigus, but it is not a cure. Even if the disorder is totally cleared, you will still be predisposed to have repeat episodes. Repeat treatments may be needed and a few individuals may require continuous therapy.

Will it spread?

Hailey-Hailey disease usually stays localized to a few selected areas of the body. A few unfortunate people get widespread involvement.

Is it contagious?

Hailey-Hailey disease is not contagious and you cannot “catch it” from anyone.