[Bullous pemphigoid].
Bullous pemphigoid is the most frequent autoimmune bullous skin disease and affects subjects who are about 80 years old. The risk factors are neurological degenerative diseases, poor Karnovski's status and some drugs (aldactone and neuroleptics). Typically, the disease consists of itching eczematous or urticarial sheets, surmounted by blisters. The blisters heal without scars. Mouth and head are rarely involved. The diagnostic is made by histological examination . It shows a subepidermal blister with some degree of dermal infiltrate with lymphocytes and eosinophils. Direct immunofluorescence reveals a linear pattern of IgG deposition along the basal membrane, which signs the diagnosis. Indirect immunofluorescence detecting anti-basal membrane antibodies is of poor diagnostic value. New tests detecting BPAg 2 antibodies by enzyme-linked immunosorbant assay (ELISA) seems to be good markers for disease activity and prognosis. Recommended treatment is topical corticosteroids (clobetasol propionate cream) for several months. It has been showed to be more effective and less dangerous than oral corticotherapy in severe forms of bullous pemphigoid. Corticosteroid sparing agents like methotrexate or mycophenolate mofetil are sometimes used because of cutaneous or systemic side effect of strong and protracted topical corticosteroid therapy. The management of these patients shall be done by specialized and coordinated staff in order to bring the best care.
Bénéton-Benhard
Nathalie
N
Centre hospitalier du Mans, service de dermatologie, 72000 Le Mans, France. nbeneton@ch-lemans.fr
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001324:Autoantigens / Q000276:immunology; D002990:Clobetasol / Q000627:therapeutic use; D004797:Enzyme-Linked Immunosorbent Assay; D019085:Fluorescent Antibody Technique, Direct; D005602:France / Q000453:epidemiology; D006652:Histological Techniques; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017567:Karnofsky Performance Status; D008727:Methotrexate / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D024041:Non-Fibrillar Collagens / Q000276:immunology; D010348:Patient Care Team; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D011379:Prognosis; D012307:Risk Factors; D000097805:Collagen Type XVII
D000893:Anti-Inflammatory Agents; D001324:Autoantigens; D002990:Clobetasol; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D008727:Methotrexate; D009173:Mycophenolic Acid; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII