Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults
| Autor: | J. Gottlieb, C. Pauwels, E. Regnier, Catherine Picard-Dahan, Marina Alexandre, Edouard Begon, C. Bernardeschi, Sophie Hue, Saskia Ingen-Housz-Oro, Olivier Chosidow, S. Grootenboer-Mignot, K. Cury, P. Schneider, Nicolas Ortonne, F. Aucouturier, Catherine Prost-Squarcioni, E. Tancrede, Frédéric Caux, Pierre Wolkenstein, Emilie Sbidian |
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| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
Adult Male medicine.medical_specialty Linear IgA bullous dermatosis Adolescent Immunoelectron microscopy Biopsy Dermatology Gastroenterology 030207 dermatology & venereal diseases 03 medical and health sciences Young Adult 0302 clinical medicine Recurrence Risk Factors Internal medicine Medicine Humans Microscopy Immunoelectron Direct fluorescent antibody Aged Retrospective Studies Skin Aged 80 and over Mucous Membrane medicine.diagnostic_test business.industry Histology IIf Middle Aged medicine.disease Lamina lucida Prognosis Linear IgA Bullous Dermatosis 030104 developmental biology Chronic Disease Disease Progression Lamina densa Female business |
| Zdroj: | The British journal of dermatology. 177(1) |
| ISSN: | 1365-2133 |
| Popis: | 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 (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. |
| Databáze: | OpenAIRE |
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