Hailey-hailey disease: an autosomal genetic disorder

Hailey-Hailey disease (HHD), also termed as familial benign chronic pemphigus, is a rare autosomal dominant blistering skin disease with waxing and waning in its clinical course. It is described by the presence of flaccid vesiculo-pustules, crusted erosions or expanding plaques in the areas of friction such as neck, axilla, groins, and perineum. Histopathologically exhibits suprabasal separations, inconspicuous dyskeratosis, acantholytic cells within the epidermis, giving a dilapidated brick wall appearance. This article impart about the clinical and histopathological features of HHD and various differential diagnosis for this disease.