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Chronic familial benign pemphigus. 1994

V N Sehgal, and S Jain
Department of Dermatology and Venereology, University College of Medical Sciences and Associated GTB Hospital, Delhi, India.

UI MeSH Term Description Entries
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D016506 Pemphigus, Benign Familial An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and BULLAE mainly on the neck, axillae, and groin. Mutations in the ATP2C1 gene (encoding the secretory pathway Ca2++/Mn2++ ATPase 1 (SPCA1)) cause this disease. It is clinically and histologically similar to DARIER DISEASE - both have abnormal, unstable DESMOSOMES between KERATINOCYTES and defective CALCIUM-TRANSPORTING ATPASES. It is unrelated to PEMPHIGUS VULGARIS though it closely resembles that disease. Chronic Benign Familial Pemphigus,Familial Benign Chronic Pemphigus,Hailey-Hailey Disease,Benign Chronic Pemphigus,Benign Familial Pemphigus,Familial Pemphigus, Benign,Hailey Hailey Disease

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