Familial benign chronic pemphigus (Hailey-Hailey disease)

A 57-year-old woman presented with a 27-year history of vesicles and crusted erosions of the intertriginous folds. Prior treatments had included topical glucocorticoids, mupirocin, and minocycline, all of which provided minimal relief. A skin biopsy specimen was consistent with Hailey-Hailey disease...

Full description

Saved in:
Bibliographic Details
Published in:Dermatology online journal 2009-08, Vol.15 (8), p.15-15
Main Authors: Warycha, Melanie, Patel, Rishi, Meehan, Shane, Merola, Joseph F
Format: Article
Language:English
Subjects:
Female
Humans
Middle Aged
Pemphigus, Benign Familial - pathology
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:A 57-year-old woman presented with a 27-year history of vesicles and crusted erosions of the intertriginous folds. Prior treatments had included topical glucocorticoids, mupirocin, and minocycline, all of which provided minimal relief. A skin biopsy specimen was consistent with Hailey-Hailey disease. Hailey-Hailey disease is the result of mutations in the ATP2C1 gene and is characterized by recurrent vesicles and erosions in intertriginous areas. Whereas topical and/or systemic antibiotic/antifungal agents in combination with topical glucocorticoids is the mainstay of treatment, case reports have documented dramatic improvement with oral retinoids, calcitriol, and tacrolimus.
ISSN:1087-2108
1087-2108
DOI:10.5070/D35X694103