Benign Familial Pemphigus (Hailey Hailey Disease)

Hailey-Hailey disease (HHD; OMIM 169600), also known as benign familial pemphigus, is a rare genodermatoses firstly described in 1939 by the Hailey brothers. HHD is characterized by impaired keratinocyte adhesion resulting in widespread intraepidermal acantholysis. Clinically, lesions appear as vesicles and bullae forming upon rupture, painful erosions, and rhagades in flexural areas. HHD is a chronic disease with a relapsing-remitting clinical course.   Exacerbations are mainly triggered by sweating, minor trauma, and secondary infections. No curative treatment is available. Patient management can be challenging. Mild cases can be controlled successfully with intermittent courses of topical corticosteroids and antibiotics.
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