title: Clinical and immunological features and outcome of anti-p200 pemphigoid.
abstract: BACKGROUND
  Anti-p200 pemphigoid is a rare autoimmune blistering disease (AIBD) of the dermoepidermal junction, characterized by autoantibodies to laminin γ1. The clinical course of anti-p200 pemphigoid in patients remains poorly investigated.
  
  
  OBJECTIVE
  We aimed to describe the clinical and immunological features and the course of a series of patients with anti-p200 pemphigoid.
  
  
  METHODS
  We conducted a retrospective study by immunoblotting detection of sera on 200-kDa dermal protein extracts from the register of the French reference centre for AIBD. We recorded the clinical and immunological features and the course of patients.
  
  
  RESULTS
  A total of 14 patients with a mean age 81·6 ± 6·5 years were included. Only one patient had an associated neurological condition and one had psoriasis. Twelve patients had atypical clinical presentation, including eczematous, urticarial, prurigo-like, dyshydrosis-like and rosette-like skin lesions. Eight patients (57%) had mucosal involvement. Immunoblot analysis of sera on dermal and epidermal extracts showed a 200-kDa band in 14 and 10 cases, respectively. All eight of the sera tested by enzyme-linked immunosorbent assay detected recombinant human laminin γ1. Disease control was obtained in six of nine patients treated with topical corticosteroids, and four of five patients who received systemic treatment. Seven patients relapsed (50%) and five patients (36%) died during the median follow-up time of 12·6 months. At the end of the study, only one of the nine living patients was in complete remission off therapy.
  
  
  CONCLUSIONS
  Many patients with anti-p200 pemphigoid had heterogeneous clinical presentation and a more severe prognosis than previously suspected.
authors.affiliation: Department of Dermatology, Rouen University Hospital, Rouen, France.|INSERM U 905, Normandy University, Rouen, France.
authors.forename: M-H
authors.initials: MH
authors.lastname: Commin
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: E
authors.initials: E
authors.lastname: Schmidt
authors.affiliation: Department of Dermatology, Rouen University Hospital, Rouen, France.
authors.forename: S
authors.initials: S
authors.lastname: Duvert-Lehembre
authors.affiliation: Department of Dermatology, Catholic Hospital Group of Lille, Lille, France.
authors.forename: A
authors.initials: A
authors.lastname: Lasek
authors.affiliation: Department of Dermatology, Caen University Hospital, Caen, France.
authors.forename: C
authors.initials: C
authors.lastname: Morice
authors.affiliation: Department of Dermatology, Desgenettes Military Hospital, Lyon, France.
authors.forename: J-L
authors.initials: JL
authors.lastname: Estival
authors.affiliation: Department of Dermatology, Lyon-South University Hospital, Lyon, France.
authors.forename: S
authors.initials: S
authors.lastname: Debarbieux
authors.affiliation: Department of Dermatology, Aurillac General Hospital, Aurillac, France.
authors.forename: E
authors.initials: E
authors.lastname: Rigal
authors.affiliation: Department of Dermatology, Toulouse University Hospital, Toulouse, France.
authors.forename: C
authors.initials: C
authors.lastname: Pauwels
authors.affiliation: Department of Dermatology, Annecy General Hospital, Annecy, France.
authors.forename: J
authors.initials: J
authors.lastname: De Quatrebarbes
authors.affiliation: Department of Dermatology, Orléans Regional Hospital, Orléans, France.
authors.forename: A
authors.initials: A
authors.lastname: Roussel
authors.affiliation: Department of Dermatology, Chalon sur Saône General Hospital, Chalon sur Saône, France.
authors.forename: E
authors.initials: E
authors.lastname: Goujon
authors.affiliation: Department of Dermatology, Nimes University Hospital, Nimes, France.
authors.forename: P-E
authors.initials: PE
authors.lastname: Stoebner
authors.affiliation: INSERM U 905, Normandy University, Rouen, France.|Laboratory of Immunology, Rouen University Hospital, Rouen, France.
authors.forename: F
authors.initials: F
authors.lastname: Jouen
authors.affiliation: Department of Dermatology, Rouen University Hospital, Rouen, France. pascal.joly@chu-rouen.fr.
authors.forename: P
authors.initials: P
authors.lastname: Joly
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D003879:Dermatologic Agents / Q000627:therapeutic use; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007797:Laminin / Q000276:immunology*; D008297:Male; D010391:Pemphigoid, Bullous / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies
chemical_list: D003879:Dermatologic Agents; D007797:Laminin; C089141:laminin gamma 1