Hailey-Hailey Disease

Several times a year a rash erupts on the chest, axillae, and neck of a 41-year-old woman. Her father and siblings have a similar history. A biopsy of the affected skin confirmed the suspected diagnosis of benign familial pemphigus, which is also called Hailey-Hailey disease. Joe Monroe, PA-C, of Tulsa, Okla, comments that this uncommon condition is usually transmitted as an autosomal dominant trait with variable penetrance. It typically presents with vesicles, erosions, and crusting caused by a loss of cohesion between the cells in the epidermis. The disorder has a predilection for intertriginous areas, such as the axillae, groin, and neck; however, outbreaks can occur elsewhere on the trunk and extremities. It is often misdiagnosed as a yeast infection. As with any blistering skin disease, a careful history, examination, and skin biopsy are necessary to make the correct diagnosis. Hailey-Hailey disease can be a challenge to treat. Topical and systemic corticosteroids; oral antibiotics, such as doxycycline(; and carbon dioxide laser ablation have been tried with mixed success. This patient’s rash showed some improvement after doxycycline and intralesional triamcinolone( were used.