ABSTRACT

Hailey-Hailey disease is also known as benign familial pemphigus. In the third or fourth decade, benign familial pemphigus usually occurs, although it can occur at any age. Then it continues to persist life-long. People of all races may be affected by it. Hailey-Hailey disease is caused by ATP2C1 gene mutations and is inherited in an autosomal dominant manner, although rare cases occur occasionally without a family history. Benign familial pemphigus typically begins as a symmetrical painful erosive and crusted skin rash in the skin folds. Diagnosis may require a skin biopsy. This systematic review aimed to collect the early and recently published research articles including PubMed and Google Scholar that confirmed the epidemiology, etiology, pathogenesis, pathophysiology, clinical features, diagnosis, treatment, management and life style modifications of Hailey–Hailey disease. There is no cure for Hailey-Hailey disease. Treatment is aimed at reducing symptoms and preventing flares. Sunlight, heat, sweating and friction often aggravate the disorder. Keywords: Hailey–Hailey disease, Desmosomes, ATP2C1 gene, Skin grafts, Botulinum toxin
Publication date: 01/10/2021

https://ijbpas.com/pdf/2021/October/MS_IJBPAS_2021_5685.pdf

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https://doi.org/10.31032/IJBPAS/2021/10.10.5685