title: Is photodynamic therapy a relevant therapeutic option in refractory benign familial pemphigus (Hailey-Hailey disease)? A series of eight patients.
abstract: BACKGROUND
  Treatment of benign familial pemphigus or Hailey-Hailey disease (HHD), a rare inherited condition associated with a significant impairment of quality of life, is often challenging and disappointing with frequent relapses and infectious complications. Topical photodynamic therapy (PDT) may offer new perspectives in this difficult setting.
  
  
  METHODS
  Eight patients with long-lasting HHD lesions refractory to multiple treatments were treated on at least one involved site with PDT using methyl-amino levulinate with a standardized protocol of three sessions of irradiation separated by 3-week intervals.
  
  
  RESULTS
  A complete or partial clearing was achieved in all treated areas, and the result was satisfactorily maintained in all cases after a follow-up period ranging from 3 to 36 months. Results were of higher quality in non-inguinal areas. Tolerance was overall acceptable with local pain during and shortly after irradiation being the main limiting factor.
  
  
  CONCLUSIONS
  Our series, although limited in size, emphasizes the interest of PDT in this difficult condition even though results may be incomplete. Treatment-related pain can be adequately managed by prior analgesics, cooling with sprayed water and local tumescent anesthesia. Overall, PDT appears as a relevant option in refractory HHD management with a favorable benefit/risk ratio.
authors.affiliation: a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
authors.forename: Maha
authors.initials: M
authors.lastname: Alsahli
authors.affiliation: a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
authors.forename: Anca
authors.initials: A
authors.lastname: Debu
authors.affiliation: a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
authors.forename: Celine
authors.initials: C
authors.lastname: Girard
authors.affiliation: a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
authors.forename: Didier
authors.initials: D
authors.lastname: Bessis
authors.affiliation: a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Du Thanh
authors.affiliation: a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
authors.forename: Bernard
authors.initials: B
authors.lastname: Guillot
authors.affiliation: a Department of Dermatology and INSERM , University of Montpellier , Montpellier , France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Dereure
mesh_terms: D000328:Adult; D000368:Aged; D004361:Drug Tolerance; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007982:Levulinic Acids / Q000627:therapeutic use*; D008027:Light; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000473:pathology; D016506:Pemphigus, Benign Familial / Q000188:drug therapy*; D010778:Photochemotherapy; D017319:Photosensitizing Agents / Q000627:therapeutic use*; D011788:Quality of Life; D016896:Treatment Outcome
chemical_list: D007982:Levulinic Acids; D017319:Photosensitizing Agents; C119867:methyl levulinate
keywords: Hailey–Hailey disease; methyl-aminolevulinate; photodynamic therapy