J Gottlieb1,2, S Ingen-Housz-Oro1,2,3, M Alexandre2,4, S Grootenboer-Mignot2,5, F Aucouturier2,6, E Sbidian1,3,7, E Tancrede2,8, P Schneider2,8,9, E Regnier2,10, C Picard-Dahan2,11, E Begon12, C Pauwels13, K Cury2,14, S Hüe2,3,15, C Bernardeschi2,9, N Ortonne2,3,16, F Caux2,4,17, P Wolkenstein1,2,3, O Chosidow1,2,3,7, C Prost-Squarcioni2,4,17,18. 1. Dermatology Department, APHP, Henri-Mondor Hospital, Créteil, France. 2. Referral Center for Autoimmune Blistering Diseases, Île-de-France, France. 3. Université Paris-Est Créteil Val de Marne, UPEC, DHU VIC, IRM, EA 7379 EpiDermE, Créteil, France. 4. Dermatology Department, APHP, Avicenne Hospital, Bobigny, France. 5. Department of Autoimmunity and Hypersensitivity, APHP, Bichat Hospital, Paris, France. 6. Immunology Department, APHP, Saint-Louis Hospital, Paris, France. 7. Inserm, Centre d'Investigation Clinique 1430, Créteil, France. 8. Dermatology Department, APHP, Saint-Louis Hospital, Paris, France. 9. Pathology Department, APHP, Saint-Louis Hospital, Paris, France. 10. Dermatology Department, APHP, Tarnier Hospital, Paris, France. 11. Dermatology Department, APHP, Bichat Hospital, Paris, France. 12. Dermatology Department, René-Dubos Hospital, Pontoise, France. 13. Dermatology Department, Saint-Germain Hospital, Saint-Germain, France. 14. Dermatology Department, APHP, Tenon Hospital, Paris, France. 15. Immunology Department, APHP, Henri-Mondor Hospital, Créteil, France. 16. Pathology Department, APHP, Henri-Mondor Hospital, Créteil, France. 17. Université Paris 13, Bobigny, France. 18. Pathology Department, APHP, Avicenne Hospital, Bobigny, France.
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. 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.
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.
Authors: Aniek Lamberts; H Ilona Euverman; Jorrit B Terra; Marcel F Jonkman; Barbara Horváth Journal: Front Immunol Date: 2018-02-19 Impact factor: 7.561