title: Hailey-Hailey disease and review of management.
abstract: OBJECTIVE
  Hailey-Hailey disease, or familial benign chronic pemphigus, is a rare genodermatosis that can be challenging for both patients and dermatologists as the disease can significantly impact patients' quality of life and is often difficult to control. In recalcitrant cases, multiple treatment modalities are often needed to obtain benefit. Unfortunately, most of the available evidence pertaining to treatment is scattered across case reports and retrospective analyses.
  
  
  OBJECTIVE
  To review successful treatments of Hailey-Hailey, synthesize the evidence, and provide recommendations for therapy.
  
  
  RESULTS
  The best evidence exists for treatment with topical steroids and topical antimicrobials. Refractory disease has shown the most benefit with addition of oral antibiotics, excisional procedures and botulinum toxin A. Other therapies are described but with much less supporting evidence.
  
  
  CONCLUSIONS
  Herein we review the literature to identify successful treatments for Hailey-Hailey disease. We have outlined the treatments with the most evidence. The difficult nature of treating this disease requires that clinicians approach each patient differently. The literature shows that no one regiment works for all patients.
authors.forename: Anthony
authors.initials: A
authors.lastname: Chiaravalloti
authors.forename: Michael
authors.initials: M
authors.lastname: Payette
mesh_terms: D000279:Administration, Cutaneous; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D019274:Botulinum Toxins, Type A / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003879:Dermatologic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D016506:Pemphigus, Benign Familial / Q000473:pathology / Q000628:therapy*; D011788:Quality of Life; D013256:Steroids / Q000008:administration & dosage
chemical_list: D000900:Anti-Bacterial Agents; D003879:Dermatologic Agents; D013256:Steroids; D019274:Botulinum Toxins, Type A