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Management of familial benign chronic pemphigus

Authors Arora H, Bray F, Cervantes J, Falto-Aizpurua L

Received 20 January 2016

Accepted for publication 29 June 2016

Published 14 September 2016 Volume 2016:9 Pages 281—290

DOI https://doi.org/10.2147/CCID.S89483

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Yan Wang

Peer reviewer comments 2

Editor who approved publication: Dr Jeffrey Weinberg


Harleen Arora, Fleta N Bray, Jessica Cervantes, Leyre A Falto Aizpurua

Department of Dermatology and Cutaneous Surgery, University of Miami-Miller School of Medicine, Miami, FL, USA

Abstract: Benign familial chronic pemphigus or Hailey–Hailey disease is caused by an ­autosomal dominant mutation in the ATP2C1 gene leading to suprabasilar acantholysis. The disease most commonly affects intertriginous areas symmetrically. The chronic nature of the disease and multiple recurrences make the disease bothersome for patients and a treatment challenge for physicians. Treatments include topical and/or systemic agents and surgery including laser. This review summarizes the available treatment options.

Keywords: familial benign chronic pemphigus, corticosteroids, dermabrasion, Hailey–Hailey disease, laser

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