Familial Benign Pemphigus – Hailey and Hailey Disease
Familial benign pemphigus (Hailey-Hailey disease) is a rare, autosomal dominant disorder with onset in adolescence or in early adulthood. Lesions are typically located in the axillae, groin, inframmary folds, and neck. Clinically, patients present with recurrent blisters, warty papules, vegetative lesions, and erosions with a characteristic fissured appearance. Involved areas tend look serpiginous due to peripheral spread of the lesions. The skin changes are uncomfortable and are exacerbated by friction, heat and pressure. They are often secondarily infected and malodorous. Mucosal surfaces are rarely involved. Nail involvement with longitudinal white streaking is common.
Light microscopy reveals intraepidermal separation and acantholysis. Both direct and indirect immunofluorescence are negative. The disorder is thought to be due to abnormalities in cellular adhesion. Electron microscopic exam demonstrates abnormalities in the desmosomal complex as well as a decreased number of desmosomes on cell surfaces.