title: Subepidermal autoimmune bullous skin diseases associated with B-cell lymphoproliferative disorders.
abstract: The development of several types of autoimmune disorders is well known in the course of B-cell lymphoproliferative diseases. In order to investigate whether these autoimmune diseases may target cutaneous antigens, especially those of the dermal-epidermal junction, we conducted a retrospective analysis of all cutaneous direct immunofluorescence studies performed in patients with B-cell neoplasia who presented with cutaneous lesions requiring a skin biopsy. Patients with linear deposits of immunoglobulin or C3 molecules at the dermal-epidermal junction were studied further, using immunoblotting and immunoelectron microscopy. Patients with no overt hematologic malignancies but with cutaneous lesions requiring direct immunofluorescence were studied as a control population. During the study period, the diagnosis of autoimmune blistering diseases of the dermal-epidermal junction was established in 9/102 (8.9% +/- 2%) patients with a B-cell lymphoid disorder, compared with 288/8,189 (3.5% +/- 0.04%) patients without underlying hemopathy (p < 0.01). Among the 9 patients, 7 were studied in detail. Immunologic studies demonstrated epidermolysis bullosa acquisita in 6 patients and cicatricial pemphigoid in 1. Autoimmune blistering diseases, especially epidermolysis bullosa acquisita, are part of the spectrum of autoimmune disorders associated with B-cell neoplasia.
authors.affiliation: Unité de Dermatologie, Hôpital Tenon, Paris, France. selim.aractingi@tnn.ap-hop-paris.fr
authors.forename: S
authors.initials: S
authors.lastname: Aractingi
authors.forename: C
authors.initials: C
authors.lastname: Bachmeyer
authors.forename: C
authors.initials: C
authors.lastname: Prost
authors.forename: F
authors.initials: F
authors.lastname: Caux
authors.forename: B
authors.initials: B
authors.lastname: Flageul
authors.forename: J P
authors.initials: JP
authors.lastname: Fermand
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D001402:B-Lymphocytes; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D015151:Immunoblotting; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D012189:Retrospective Studies; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology* / Q000276:immunology / Q000473:pathology