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Resume Introduction La pemphigoide anti-p200 est une dermatose bulleuse auto-immune de description recente caracterisee par sa cible antigenique et son tableau anatomoclinique. Son traitement est encore mal codifie. Observation Un homme de 73 ans consultait pour une eruption bulleuse et prurigineuse associee a une atteinte muqueuse. L'immunofluorescence directe cutanee (IFD) montrait un depot lineaire d'IgG et de C3 le long de la jonction dermo-epidermique (JDE). La recherche d'anticorps anti-BP180 et anti-BP230 par Elisa etait negative. Le diagnostic de pemphigoide bulleuse etait evoque. Apres une evolution clinique defavorable sous traitement par clobetasol topique (Dermoval®), puis prednisolone et methotrexate, de nouveaux examens immunologiques etaient realises. L'immunofluorescence indirecte (IFI) sur peau clivee montrait un marquage dermique par l'IgG4. L'immuno-empreinte sur extrait dermique mettait en evidence une bande migrant a 200 kDa. Le diagnostic de pemphigoide anti-p200 etait alors retenu. Le patient etait traite par dapsone associee a la prednisolone. A 72 heures, une cytolyse hepatique evocatrice d'hepatite immuno-allergique faisait interrompre le traitement. Secondairement, un traitement par mycophenolate mofetil etait instaure, permettant une remission complete maintenue a sept mois. Discussion Un ensemble d'elements cliniques et immunologiques a fait retenir le diagnostic de pemphigoide anti-p200 chez notre patient. La pemphigoide anti-p200 se differencie de la pemphigoide bulleuse par une atteinte cephalique, acrale et muqueuse plus frequente et la cicatrisation avec grains de milium. Il n'y a pas d'hypereosinophilie et les anticorps anti-BP180 et anti-BP230 sont negatifs en Elisa. L'immuno-empreinte permet de faire le diagnostic en mettant en evidence des anticorps diriges contre une proteine de 200 kDa sur l'extrait dermique. Le traitement est encore mal defini, meme si la dapsone semble le traitement le plus efficace. A notre connaissance, notre patient est le premier cas decrit traite avec efficacite par mycophenolate mofetil. Conclusion La prise en charge therapeutique de la pemphigoide anti-p200 est difficile. Chez notre patient, le traitement par mycophenolate mofetil a ete efficace et semble une alternative a la dapsone. ________________________________________ Summary Background Anti-p200 pemphigoid is a recently described autoimmune subepidermal bullous dermatosis characterized by its target antigen and the associated anatomoclinical picture. The treatment is not as yet well defined. Patient and methods A 73-year-old man consulted for a pruritic bullous eruption with buccal involvement. Direct immunofluorescence revealed linear deposits of IgG and C3 at the dermal-epidermal junction. Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. A diagnosis of bullous pemphigoid was suspected. After an unfavourable clinical outcome under clobetasol and then prednisolone and methotrexate, other immunological tests were performed. Indirect immunofluorescence on NaCl-cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting showed antibodies directed against a 200-kDa antigen on a dermal extract. A diagnosis of anti-p200 pemphigoid was made. The patient was treated with dapsone combined with prednisolone. Seventy-two hours later, treatment was stopped due to hepatic cytolysis related to immunoallergic hepatitis. Treatment with mycophenolate mofetil was then initiated and resulted in complete remission, which persisted at seven months. Discussion The diagnosis of anti-p200 pemphigoid was made on the basis of a set of clinical and immunological factors. Anti-p200 pemphigoid differs from standard bullous pemphigoid in terms of more frequent cephalic, acral and mucous membrane involvement, as well as a greater degree of miliary scarring. There was no eosinophilia. Elisa screening for anti-BP180 and anti-BP230 antibodies was negative. Immunoblotting showed antibodies directed against a 200 kDa protein on dermal extract. The treatment is not well defined, even if dapsone appears to be the most effective therapy. To our knowledge, our patient is the first to be successfully treated with mycophenolate mofetil. Conclusion Treatment of anti-p200 pemphigoid is difficult. In our case, treatment by mycophenolate mofetil was effective and could offer an alternative to dapsone. Mots cles " Pemphigoide anti-p200; " Pemphigoide bulleuse; " Traitement; " Mycofenolate mofetil Keywords " Anti-p200 pemphigoid; " Bullous pemphigoid; " Treatment; " Mycofenolate mofetil ________________________________________ Figures and tables from this article: Figure 1. Plaques erythemateuses, elements purpuriques et figures, erosions post-bulleuses. Figure options Figure 2. Erosions post-bulleuses acrales. Figure options Figure 3. Erosions linguales et labiales. Figure options Tableau 1. Comparaison clinique, histologique et immunologique entre la pemphigoide bulleuse et la pemphigoide anti-p200. IFD : immunofluorescence directe cutanee ; JDE : jonction dermo-epidermique ; IFI : immunofluorescence indirecte ; PB : pemphigoide bulleuse. |
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