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Hailey-Hailey disease

Also known as benign familial pemphigus

Hailey-Hailey is not an autoimmune disease and it is often genetic in nature. The majority of the time, it is seen in family medical histories and is often seen intergenerationally. Siblings and extended family members are often affected together. It is frequently misdiagnosed but not life-threatening.

Onset is often in the late teens or in the third and fourth decade of life. It is seen equally in men and women. Attacks of HH are often frequent with short periods of remission. The disease usually lessens with age

The most commonly affected areas are the arms and legs, the groin and where skin rubs against itself. Significant discomfort from burning and itch can occur which may restrict work and leisure activities. Heat, friction and infection exacerbate outbreaks so patients are urged to maintain option weight to lessen skin friction, and to wear cool and loose fitting garments.

Treatment consists of oral antibiotics to control infection, cool compresses and dressings. Topical steroid creams have been useful in some cases. Oral steroids and Dapsone are sometimes effective in severe breakouts. The disease was first described in 1939.

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