Methotrexate for refractory Hailey–Hailey disease

Editor We would like to report the use of methotrexate to treat a female patient with refractory, long standing Hailey–Hailey disease (HHD). To our knowledge, this is only the second case report of methotrexate being used to treat this disease. A 42-year-old woman presented to us with active skin lesions affecting both axillae, groins and lateral aspect of neck (Fig. 1) that had an ondulant course, with acute exacerbations and partial remissions for 10 years. A positive family history was present. Skin biopsy confirmed the clinical diagnosis of Hailey–Hailey disease (Fig. 2). Previous treatments included mainly topical corticosteroids, and topical and oral courses of antibiotics and antifungals. Considering disease extension and also standard treatment failure, we decided to start oral methotrexate, 15 mg per week. Lesions started to improve and were nearly clear 1 month later, leaving postinflammatory hyperpigmentation (Fig. 3). Methotrexate was tapered and finally stopped 2 months later. She remained asymptomatic since then with 2 years follow-up and topical preparation of betamethasone/fucidic acid is sufficient to abort discrete developing lesions.