Hailey - Hailey Disease - A Case Report

This case report emphasizes the importance of a multidisciplinary approach and provides valuable insights into the clinical course and management strategies for Hailey-Hailey disease. Hailey-Hailey disease is a rare autosomal dominant skin disorder characterized by recurrent blistering and erosions primarily affecting intertriginous areas. We present a case report of a 42-year old male patient with a confirmed diagnosis of Hailey-Hailey disease. The patient had a history of pruritic skin lesions over neck, inframammary areas since the age of 18. The lesions aggravated more during summer than winter. Clinical examination revealed discrete vesicles, erosions, macerations present in the bilateral inframammary areas, flexures and scalp along with crusting. Histopathological examination of skin biopsy taken from right inframammary area revealed supra basal vesiculobullous lesion. Treated with systemic antibacterial, topical antifungals to cover for secondary infection following topical steroids. The skin lesions improved. Then the patient came with complaints of itching & skin lesions on scalp, trunk, groin. At first tab Dapsone 50 mg was started, but the patient was not tolerated and hence it was stopped. Therefore, she was treated with oral antibiotics, topical steroids, antihistamines. Eventually, skin lesions were reducing and improving. Now, in remission and on medications. This case report provides valuable resources of unusual information that may lead to research and advances in clinical practice.