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Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults
Author(s) -
Gottlieb J.,
IngenHouszOro S.,
Alexandre M.,
GrootenboerMignot S.,
Aucouturier F.,
Sbidian E.,
Tancrede E.,
Schneider P.,
Regnier E.,
PicardDahan C.,
Begon E.,
Pauwels C.,
Cury K.,
Hüe S.,
Bernardeschi C.,
Ortonne N.,
Caux F.,
Wolkenstein P.,
Chosidow O.,
ProstSquarcioni C.
Publication year - 2017
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.15244
Subject(s) - medicine , iif , pathology , univariate analysis , gastroenterology , autoantibody , antibody , immunology , multivariate analysis
Summary 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. Objectives 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 ( IB s) 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 ( AF z) (19%) and LL + AF z (23%). Of the 34 IgA IB s, 22 were positive, mostly for LAD ‐1/ LABD 97 (44%) and full‐length BP 180 (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.