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Esperienze Dermatologiche 2012 December;14(4):163-5

Copyright © 2013 EDIZIONI MINERVA MEDICA

language: Italian

Familial chronic pemphigus (Hailey-Hailey disease)

Nazzaro G., Pesapane F., Coggi A., Gianotti R.

Sezione di Scienze Dermatologiche, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milano, Italia


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The Hailey-Hailey disease, or “familial chronic pemphigus”, is a rare autosomal dominant disease with incomplete penetrance, which manifests clinically with fragile vesicles/blisters and recurrent erosions in intertriginous regions and histologically with a suprabasal acantholysis. We describe the case of a 33 year old woman presenting a single submammary lesion since about 8 months; no family medical history was reported. The histological examination showed a “dilapidated brick wall” appearance, typical for pemphigus familiaris benignus chronicus and immunofluorescence was negative.

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