title: Epidermolysis bullosa acquisita: a retrospective analysis of 12 patients evaluated in four tertiary hospitals in Spain.
abstract: BACKGROUND
  Epidermolysis bullosa acquisita is an exceedingly rare subepidermal blistering disease caused by antibodies against type VII collagen. Studies summarizing the clinical and immunological features of this disease in large series of patients are scarce.
  
  
  OBJECTIVE
  To analyse the clinical and immunopathological characteristics, treatment responses and outcomes of 12 patients with epidermolysis bullosa acquisita from four tertiary hospitals in Spain.
  
  
  METHODS
  An extensive retrospective review of clinical charts.
  
  
  RESULTS
  The mean age of onset was 48 years and the mean delay to diagnosis was 20·75 months. The classical phenotype occurred in 42% of cases, inflammatory in 42% and mixed in 17%. Mucosal involvement was present in 75%. Linear IgG deposition along the basement membrane zone was consistently present on direct immunofluorescence examination. Indirect immunofluorescence study was positive in 67% of the cases. Frequently associated diseases were neoplasms (25%), inflammatory bowel disease (25%), hepatitis C virus infection (17%) and thyroid dysfunction (17%). Therapeutic responses were variable.
  
  
  CONCLUSIONS
  The prevalence of neoplasms was similar to that seen in inflammatory bowel disease. Multicentric prospective studies including larger numbers of patients are required for a better knowledge and management of this disease.
authors.affiliation: Dermatology Department, Hospital Clínic, Villarroel 170, 08036, Barcelona, Spain.
authors.forename: P
authors.initials: P
authors.lastname: Iranzo
authors.forename: J E
authors.initials: JE
authors.lastname: Herrero-González
authors.forename: J M
authors.initials: JM
authors.lastname: Mascaró-Galy
authors.forename: R
authors.initials: R
authors.lastname: Suárez-Fernández
authors.forename: A
authors.initials: A
authors.lastname: España
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000378:metabolism; D057210:Delayed Diagnosis; D003879:Dermatologic Agents / Q000627:therapeutic use; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006760:Hospitalization; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013030:Spain; D062606:Tertiary Care Centers; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000974:Antibodies, Antinuclear; D003879:Dermatologic Agents