[Bullous pemphigoid].
Bullous pemphigoid (BP) is the most frequent autoimmune blistering skin disease of the elderly. It is mediated by circulating antibodies directed against two hemidesmosomal proteins of the dermal epidermal junction: BPAG1 and BPAG2. Clinical features consist of pruritus and tense blisters usually surrounded by erythema. Blisters sometimes evolve to erosions, become haemorrhagic or even large erosive areas. Lesions heal without scarring. Lesions are symmetrically located on the thighs, legs, trunck and arms. Mucous membranes are usually uninvolved. Histological examination of a skin biopsy specimen shows a subepidermal blister with eosinophils within the blister and the superficial dermis. Direct immunofluorescence shows linear IgG and/or C3 deposits along the dermal epidermal junction. In France and in Europe, most patients are now treated using topical steroid therapy (clobetasol propionate).
de Quatrebarbes
Julie
J
Clinique dermatologique, hôpital Charles Nicolle, 76000 Rouen. Julie.de-quatrebarbes@chu-rouen.fr
Joly
Pascal
P
D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001324:Autoantigens / Q000032:analysis; D002352:Carrier Proteins / Q000032:analysis; D002990:Clobetasol / Q000031:analogs & derivatives / Q000627:therapeutic use; D003598:Cytoskeletal Proteins / Q000032:analysis; D000071821:Dystonin; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D009419:Nerve Tissue Proteins / Q000032:analysis; D024041:Non-Fibrillar Collagens / Q000032:analysis; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D000097805:Collagen Type XVII
D000893:Anti-Inflammatory Agents; D001324:Autoantigens; D002352:Carrier Proteins; D002990:Clobetasol; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D007074:Immunoglobulin G; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human