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Case Report • An. Bras. Dermatol. 95 (1) • Jan-Feb 2020 • https://doi.org/10.1016/j.abd.2019.02.006   copy

Familial “benign” pemphigus? Erythroderma and fatal outcome☆ ☆ How to cite this article: Tansini PB, Boff AL, Weber MB, Bonamigo RR. Familial “benign” pemphigus? Erythroderma and fatal outcome. An Bras Dermatol. 2019;95:75-7. ,☆☆ ☆☆ Study conducted at the Public Health Dermatology Service, Secretaria Estadual de Saúde, Porto Alegre, RS, Brazil.

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Abstract

Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

KEYWORDS
Pemphigus, benign familial; Dermatitis, exfoliative; Skin diseases, vesiculobullous

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