title: Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults.
abstract: BACKGROUND
  Linear IgA bullous dermatosis (LABD) is a clinically and immunologically heterogeneous, subepidermal, autoimmune bullous disease (AIBD), for which the long-term evolution is poorly described.
  
  
  OBJECTIVE
  To investigate the clinical and immunological characteristics, follow-up and prognostic factors of adult idiopathic LABD.
  
  
  METHODS
  This retrospective study, conducted in our AIBD referral centre, included adults, diagnosed between 1995 and 2012, with idiopathic LABD, defined as pure or predominant IgA deposits by direct immunofluorescence. Clinical, histological and immunological findings were collected from charts. Standard histology was systematically reviewed, and indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblots (IBs) on amniotic membrane extracts using anti-IgA secondary antibodies were performed, when biopsies and sera obtained at diagnosis were available. Prognostic factors for complete remission (CR) were identified using univariate and multivariate analyses.
  
  
  RESULTS
  Of the 72 patients included (median age 54 years), 60% had mucous membrane (MM) involvement. IgA IIF on SSS was positive for 21 of 35 patients tested; 15 had epidermal and dermal labellings. Immunoelectron microscopy performed on the biopsies of 31 patients labelled lamina lucida (LL) (26%), lamina densa (23%), anchoring-fibril zone (AFz) (19%) and LL+AFz (23%). Of the 34 IgA IBs, 22 were positive, mostly for LAD-1/LABD97 (44%) and full-length BP180 (33%). The median follow-up was 39 months. Overall, 24 patients (36%) achieved sustained CR, 19 (29%) relapsed and 35% had chronic disease. CR was significantly associated with age > 70 years or no MM involvement. No prognostic immunological factor was identified.
  
  
  CONCLUSIONS
  Patients with LABD who are < 70 years old and have MM involvement are at risk for chronic evolution.
authors.affiliation: Dermatology Department, APHP, Henri-Mondor Hospital, Créteil, France.|Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.
authors.forename: J
authors.initials: J
authors.lastname: Gottlieb
authors.affiliation: Dermatology Department, APHP, Henri-Mondor Hospital, Créteil, France.|Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Université Paris-Est Créteil Val de Marne, UPEC, DHU VIC, IRM, EA 7379 EpiDermE, Créteil, France.
authors.forename: S
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Avicenne Hospital, Bobigny, France.
authors.forename: M
authors.initials: M
authors.lastname: Alexandre
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Department of Autoimmunity and Hypersensitivity, APHP, Bichat Hospital, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Grootenboer-Mignot
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Immunology Department, APHP, Saint-Louis Hospital, Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Aucouturier
authors.affiliation: Dermatology Department, APHP, Henri-Mondor Hospital, Créteil, France.|Université Paris-Est Créteil Val de Marne, UPEC, DHU VIC, IRM, EA 7379 EpiDermE, Créteil, France.|Inserm, Centre d'Investigation Clinique 1430, Créteil, France.
authors.forename: E
authors.identifier: http://orcid.org/0000-0002-1267-5270
authors.initials: E
authors.lastname: Sbidian
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Saint-Louis Hospital, Paris, France.
authors.forename: E
authors.initials: E
authors.lastname: Tancrede
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Saint-Louis Hospital, Paris, France.|Pathology Department, APHP, Saint-Louis Hospital, Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Schneider
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Tarnier Hospital, Paris, France.
authors.forename: E
authors.initials: E
authors.lastname: Regnier
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Bichat Hospital, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Picard-Dahan
authors.affiliation: Dermatology Department, René-Dubos Hospital, Pontoise, France.
authors.forename: E
authors.initials: E
authors.lastname: Begon
authors.affiliation: Dermatology Department, Saint-Germain Hospital, Saint-Germain, France.
authors.forename: C
authors.initials: C
authors.lastname: Pauwels
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Tenon Hospital, Paris, France.
authors.forename: K
authors.initials: K
authors.lastname: Cury
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Université Paris-Est Créteil Val de Marne, UPEC, DHU VIC, IRM, EA 7379 EpiDermE, Créteil, France.|Immunology Department, APHP, Henri-Mondor Hospital, Créteil, France.
authors.forename: S
authors.initials: S
authors.lastname: Hüe
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Pathology Department, APHP, Saint-Louis Hospital, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Bernardeschi
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Université Paris-Est Créteil Val de Marne, UPEC, DHU VIC, IRM, EA 7379 EpiDermE, Créteil, France.|Pathology Department, APHP, Henri-Mondor Hospital, Créteil, France.
authors.forename: N
authors.initials: N
authors.lastname: Ortonne
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Avicenne Hospital, Bobigny, France.|Université Paris 13, Bobigny, France.
authors.forename: F
authors.initials: F
authors.lastname: Caux
authors.affiliation: Dermatology Department, APHP, Henri-Mondor Hospital, Créteil, France.|Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Université Paris-Est Créteil Val de Marne, UPEC, DHU VIC, IRM, EA 7379 EpiDermE, Créteil, France.
authors.forename: P
authors.initials: P
authors.lastname: Wolkenstein
authors.affiliation: Dermatology Department, APHP, Henri-Mondor Hospital, Créteil, France.|Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Université Paris-Est Créteil Val de Marne, UPEC, DHU VIC, IRM, EA 7379 EpiDermE, Créteil, France.|Inserm, Centre d'Investigation Clinique 1430, Créteil, France.
authors.forename: O
authors.initials: O
authors.lastname: Chosidow
authors.affiliation: Referral Center for Autoimmune Blistering Diseases, Île-de-France, France.|Dermatology Department, APHP, Avicenne Hospital, Bobigny, France.|Université Paris 13, Bobigny, France.|Pathology Department, APHP, Avicenne Hospital, Bobigny, France.
authors.forename: C
authors.identifier: http://orcid.org/0000-0002-9197-936X
authors.initials: C
authors.lastname: Prost-Squarcioni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002908:Chronic Disease; D018450:Disease Progression; D005260:Female; D006801:Humans; D062027:Linear IgA Bullous Dermatosis / Q000473:pathology*; D008297:Male; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D009092:Mucous Membrane / Q000473:pathology; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D012867:Skin / Q000473:pathology*; D055815:Young Adult