Topical Tacrolimus Ointment Is an Effective Therapy for Hailey-Hailey Disease

Abstract

A 67-year-old man with a 10-year history of flexural blistering eruptions also affecting 3 of his brothers was first evaluated in 1987. The patient presented with vesicles, erosions, and erythema in the intertriginous areas but also as multiple truncal plaques (Figure 1). Biopsy specimens showed intraepidermal clefts of varying sizes both suprabasally and higher in the epidermis, as well as the characteristic incomplete acantholysis in large parts of the epidermis, giving it the appearance of a “dilapidated brick wall” (Figure 2). The findings of a direct immunofluorescence evaluation were negative, which is consistent with a diagnosis of Hailey-Hailey disease (or chronic benign familial pemphigus). A daily application of an ointment of betamethasone—a potent corticosteroid ointment—and clioquinol for 3 months gave no improvement in the condition. Similarly, neither topically applied clobetasol nor systemic antibiotics directed against Staphylococcus aureus had any disease-modifying effect. Then, 100 mg of dapsone daily and later 100 to 150 mg of azathioprine daily were administered successively for 3 months without significant beneficial effect. Oral daily treatment with 5 mg/kg of cyclosporine for 6 months reduced the activity of the disease, but this treatment was stopped owing to severe headache. Finally, 1% cyclosporine cream applied twice daily for 3 months did not result in healing. Because of the disseminated distribution of the lesions, carbon dioxide laser treatment was not considered an appropriate therapeutic option.