A Rare Case of Atypical Recalcitrant Hailey-Hailey Disease and a Literature Review

Abstract

Hailey-Hailey disease also known as familial benign chronic pemphigus is a rare bullous genodermatosis that affects intertriginous area symmetrically. It presents with flaccid blisters, erosions and maceration resulting in increased morbidity, reduced quality of life for affected patients. It is rare in occurrence with an incidence of rate of 1 in 50,000. It is diagnosed with a combination of clinical and histopathological findings. While there is no known cure, its relapsing remitting course can be managed with medication. This case describes an unusual presentation of familial benign chronic pemphigus with a late age of onset of symptoms, atypical distribution and resistant to multiple therapies.