Familial Benign Pemphigus (Hailey- Hailey Disease)

Familial benign pemphigus originally was described by the Hailey brothers in 1939.1 It is a chronic autosomal dominant disorder with incomplete penetrance. Approximately two thirds of patients have a family history of the disorder. A history of multiple relapses and remissions is characteristic. Decreased numbers of desmosomes have been implicated in the pathogenesis of benign familial pemphigus. Therapeutic options are limited.

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