Hailey Hailey disease-a rare case report

Abstract

Hailey-Hailey disease is an autosomal dominant acantholytic disorder relatively uncommon in India. It is characterized by painful, pruritic, foul smelling vesicles and bullous lesions with erosions in intertriginous areas such as the neck, axillae, inframammary areas and groin. The defect in ATPC2 gene leads to calcium channel dysfunction which results in defect in epidermis. Fungal infection, intertrigo, psoriasis, extramammary Paget's disease, acanthosis nigricans, pemphigus vegetans and Darier's disease are some of the dermatosis from which it needs to be differentiated. Histopathology has a key role in diagnosis of Hailey-Hailey disease with characteristic ‘dilapidated brick wall' appearance. Hailey-Hailey disease can be easily misdiagnosed by general physicians due to lack of knowledge of this uncommon disease as it resembles dermatosis involving intertriginous areas. Due to its relapsing and remitting course there is a need to have effective treatment options. We report a case of 45 year old middle aged female who presented with on and off itchy papules & vesicles over right inframammmary region around the umbilicus & medial aspect of right thigh. Skin biopsy revealed a diagnosis of Hailey-Hailey disease. This case is being reported due to its rare occurrence.