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Benign Familial Chronic Pemphigus

Autosomal dominant inherited skin disease with variable gene penetration characterized by recurrent eruptions of vesicles and bullae mainly in intertriginous areas. The neck, axillae and the inguinal or perianal region are typically involved. Lesions show itchy patches with scaling crusts and fissured surfaces. They extend peripherically and the centre may heal or show soft, flat, moist vegetations. The course is chronically recurrent with remissions. Complications due to secondary infection may occur. A number of external stimuli have been shown to produce lesions including friction, freezing and ultraviolet radiation. The disorder is unrelated to pemphigus vulgaris.

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