Successful treatment of Hailey-Hailey disease with acitretin

Hailey-Hailey disease is an autosomal dominant skin condition characterized by waxing and waning painful and pruritic vesicles and plaques affecting the intertriginous areas. Its pathogenesis involves inherited abnormalities in a cutaneous calcium pump. Most patients are managed conservatively with topical corticosteroids as well as topical and oral anti-infective agents. Scarce reports in the literature describe the use of oral retinoid therapy to manage refractory cases. We present a case of Hailey-Hailey disease in a 64-year-old man who was refractory to conservative management but improved dramatically over 6 months of oral therapy with 25 mg of acitretin daily. The mechanism by which such therapy improves disease manifestations is unknown. A potential mechanism is based on the influence of retinoids on epidermal differentiation and may involve cutaneous calcium homeostasis. Hailey-Hailey disease is discussed and the use of oral retinoid treatment for Hailey-Hailey disease is reviewed.