[Epidermolysis bullosa acquisita].
Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal bullous disease with autoimmunity to the type VII collagen which is the major component of anchoring fibrils. Clinical manifestations of the classical EBA include skin fragility, blisters over the trauma-prone surfaces and milium cysts. Other presentations of EBA have been reported: mucosal predominant appearance reminiscent of cicatricial pemphigoid, bullous pemphigoid-like presentation and IgA-EBA. Making a definitive diagnosis of EBA could be difficult because specialized tests available in only some laboratories are necessary to confirm the clinical suspicion: immunoelectron microscopy demonstrating immune deposits on anchoring fibrils and immunoblotting or enzyme-linked immunosorbent assay (Elisa) detecting autoantibodies recognizing the type VII collagen. EBA frequently is associated with Crohn's disease and an inflammatory bowel disease must be ruled out in patients with EBA and abdominal manifestations. EBA potentially is serious, has usually a chronical evolution and is difficult to treat.There are no guidelines for treatment of EBA, which is adapted to clinical severity and include dapsone, cyclosporine and rituximab.
Caux
Frédéric
F
Service de dermatologie et centre de référence des dermatoses bulleuses toxiques et auto-immunes, hôpital Avicenne, AP-HP, 125, rue de Stalingrad, 93000 Bobigny, France. frederic.caux@avc.aphp.fr
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001327:Autoimmune Diseases / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D024143:Collagen Type VII / Q000276:immunology; D003424:Crohn Disease / Q000150:complications; D016572:Cyclosporine / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D016107:Epidermolysis Bullosa Acquisita / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D005455:Fluorescent Antibody Technique; D006652:Histological Techniques; D006801:Humans; D015151:Immunoblotting; D007070:Immunoglobulin A / Q000276:immunology; D007155:Immunologic Factors / Q000627:therapeutic use; D016253:Microscopy, Immunoelectron; D017410:Practice Guidelines as Topic; D035583:Rare Diseases; D000069283:Rituximab; D012720:Severity of Illness Index
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D024143:Collagen Type VII; D007070:Immunoglobulin A; D007155:Immunologic Factors; D000069283:Rituximab; D016572:Cyclosporine; D003622:Dapsone