title: Successful treatment of Hailey-Hailey disease with acitretin.
abstract: 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.
authors.affiliation: Department of Dermatology, Tufts-New England Medical Center, Tufts University School of Medicine, Boston, MA 02111, USA. emily.berger@tufts.edu
authors.forename: Emily M
authors.initials: EM
authors.lastname: Berger
authors.forename: Hassan I
authors.initials: HI
authors.lastname: Galadari
authors.forename: Alice B
authors.initials: AB
authors.lastname: Gottlieb
mesh_terms: D017255:Acitretin / Q000008:administration & dosage / Q000627:therapeutic use*; D000284:Administration, Oral; D006801:Humans; D007641:Keratolytic Agents / Q000008:administration & dosage / Q000737:chemistry / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D016506:Pemphigus, Benign Familial / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D007641:Keratolytic Agents; D017255:Acitretin