2016•JAAD Case ReportsOpen access

Familial benign chronic pemphigus (Hailey-Hailey disease) treated with electron beam radiation

Paul M. Graham, Allison A. Melkonian, David P. Fivenson

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Abstract

Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. In this condition, dysregulation of intracellular calcium homeostasis leads to impaired desmosome function and suprabasilar acantholysis of the epidermis.1 Clinically, this condition presents as intertriginous vesicles, erosions, and weeping, fissured plaques.1 The painful nature of HHD, risk of secondary infection, and malodor can significantly affect a patient's quality of life. The lesions are often difficult to control with topical steroids and antimicrobials, necessitating more aggressive treatments.

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What this paper is about

Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. In this condition, dysregulation of intracellular calcium homeostasis leads to impaired desmosome function and suprabasilar acantholysis of the epidermis.1 Clinically, this condition presents as intertriginous vesicles, erosions, and weeping, fissured plaques.1 The painful nature of HHD, risk of secondary infection, and malodor can significantly affect a patient's quality of life. The lesions are often difficult to control with topical steroids and antimicrobials, necessitating more aggressive treatments.

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Available abstract

Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. In this condition, dysregulation of intracellular calcium homeostasis leads to impaired desmosome function and suprabasilar acantholysis of the epidermis.1 Clinically, this condition presents as intertriginous vesicles, erosions, and weeping, fissured plaques.1 The painful nature of HHD, risk of secondary infection, and malodor can significantly affect a patient's quality of life. The lesions are often difficult to control with topical steroids and antimicrobials, necessitating more aggressive treatments.

Key concepts: Hailey–Hailey disease, Intertriginous, Medicine, Acantholysis, Genodermatosis, Pemphigus, Dermatology, Disease

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