Hailey-Hailey disease: a diagnostic challenge

Abstract

Hailey-Hailey disease (HHD) is an autosomal-dominant genodermatosis characterized by crusted macerated erosions, as well as velvety, dry, fissured plaques in the intertriginous areas. No predilection for sex or ethnic group has been reported. The typical age of onset is in the third decade of life. Diagnosis of HHD is suggested based on clinical morphology, location of lesions, family history, and histology demonstrating a characteristic dilapidated brick wall appearance of the epidermis. However, HHD often is misdiagnosed due to lack of knowledge of this uncommon disorder and its resemblance to other dermatoses. We describe an unusual presentation of HHD with a late age of onset and involvement of nonintertriginous regions.