benign familial pemphigus

benign familial pemphigus
a benign, autosomal dominant dermatitis due to mutation in the ATP2C1 gene (locus: 3q21-q24), which encodes a Ca2+-ATPase. It is characterized by recurrent bullae and vesicles, usually on the neck, axillae, groin, and other flexural and apposing surfaces. Lesions are in crops and may remain localized or become generalized, rupture, erode, and develop a thick crust. The defect appears to be in keratinocyte cohesion. Called also Hailey-Hailey disease.

Medical dictionary. 2011.

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  • pemphigus — pemphigoid /pem fi goyd /, adj. pemphigous, adj. /pem fi geuhs, pem fuy /, n. Pathol. any of several diseases, often fatal, characterized by blisters on the skin and mucous membranes. [1770 80; < NL < Gk pemphig (s. of pémphix) bubble + L us n.… …   Universalium

  • familial benign pemphigus — benign familial p …   Medical dictionary

  • pemphigus — 1. Autoimmune bullous diseases with acantholysis : p. vulgaris, p. foliaceus, p. erythematosus, or p. vegetans. 2. A nonspecific term for blistering skin diseases. [G. pemphix, a blister] benign familial …   Medical dictionary

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  • Pemphigus — ou Pemphigoïde ou Maladie de la Bulle Classification et ressources externes CIM 10 L10 CIM 9 694.4 OMIM …   Wikipédia en Français

  • Benign fibrous histiocytoma — Dermatofibroma Classification and external resources ICD 10 D23 (ILDS D23.L62) ICD 9 216.9 …   Wikipedia

  • Hailey Hailey Disease (Familial Benign Pemphigus) — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 29321 ICD10 = ICD10|Q|82|8|q|80 ICD9 = ICD9|757.39 ICDO = OMIM = 169600 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = D016506 Familial benign pemphigus originally was described by… …   Wikipedia

  • Hailey-Hailey disease — benign familial pemphigus …   Medical dictionary

  • Hailey-Hailey disease — Hai·ley Hai·ley disease (haґle haґle) [Hugh Edward Hailey, American dermatologist, 1909–1964; William Howard Hailey, American dermatologist, 1898–1967] benign familial pemphigus; see under pemphigus …   Medical dictionary

  • transient acantholytic dermatosis — a self limited papulovesicular disease usually seen in middle aged persons, most often on the trunk, with histologic changes resembling those of Darier disease or benign familial pemphigus. It is sometimes an epidermal reaction to actinic injury …   Medical dictionary

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