A case of Hailey-Hailey disease in late onset presentation

Abstract

            Hailey-Hailey disease is one of the autosomal dominant genodermatoses which is caused by a mutation of the ATP2C1 gene. Patients usually present between the 2^nd and 4^th decades of life. Clinical presentations consist of blisters on the erythematous base, crusted erosion and vegetative plaques on intertriginous areas or sites of trauma.
            We report a case of a 67-year-old Thai woman with chronic erythematous crusted plaques on bilateral inguinal areas. The clinical and histopathologic findings were compatible with Hailey-Hailey disease. She first experienced the skin lesions at 59 years old which is much later than the usual age of onset of the disease. She was treated with topical steroids, and resolution was observed within 2 weeks.