title: [Bullous pemphigoid in young patients: a retrospective study of 74 cases].
abstract: BACKGROUND
  Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported.
  
  
  OBJECTIVE
  Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations.
  
  
  METHODS
  Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies.
  
  
  RESULTS
  Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy.
  
  
  CONCLUSIONS
  Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.
authors.affiliation: Service de Dermatologie, Hôpital Henri Mondor, 94010 Créteil, France
authors.forename: E
authors.initials: E
authors.lastname: Bourdon-Lanoy
authors.forename: J-C
authors.initials: JC
authors.lastname: Roujeau
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.forename: J-C
authors.initials: JC
authors.lastname: Guillaume
authors.forename: P
authors.initials: P
authors.lastname: Bernard
authors.forename: C
authors.initials: C
authors.lastname: Prost
authors.forename: E
authors.initials: E
authors.lastname: Tancrède-Bohin
authors.forename: E
authors.initials: E
authors.lastname: Delaporte
authors.forename: C
authors.initials: C
authors.lastname: Picard-Dahan
authors.forename: B
authors.initials: B
authors.lastname: Albes
authors.forename: C
authors.initials: C
authors.lastname: Bedane
authors.forename: M-S
authors.initials: MS
authors.lastname: Doutre
authors.forename: O
authors.initials: O
authors.lastname: Chosidow
authors.forename: C
authors.initials: C
authors.lastname: Lok
authors.forename: C
authors.initials: C
authors.lastname: Pauwels
authors.forename: J
authors.initials: J
authors.lastname: Chevrand-Breton
authors.forename: B
authors.initials: B
authors.lastname: Sassolas
authors.forename: M-A
authors.initials: MA
authors.lastname: Richard
mesh_terms: D000328:Adult; D017668:Age of Onset; D001323:Autoantibodies / Q000032:analysis*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012878:Skin Neoplasms / Q000209:etiology
chemical_list: D001323:Autoantibodies