Janisson-Dargaud D, Reguiaï Z, Perceau G, Eschard C, Bernard P
Service de dermatologie, hôpital Robert-Debré, CHU de Reims, avenue Général-Koenig, 51100 Reims, France.
Ann Dermatol Venereol. 2008 Dec;135(12):843-7. doi: 10.1016/j.annder.2007.11.037. Epub 2008 Aug 29.
Pemphigus vulgaris is a rare autoimmune blistering disease seen only, rarely, in children.
CASE-REPORT: Two young girls, aged four and 15 years respectively, presented with oral and/or cutaneous blisters (case 2). The diagnosis of pemphigus vulgaris was confirmed by histology (suprabasal acantholysis) and immunopathological analysis (direct and indirect immunofluorescence, antidesmoglein-3 plus or minus antidesmoglein-1 antibodies by ELISA). In case 2, caused by corticosteroid dependence and after the failure of intravenous immunoglobulin, the patient was successfully treated with rituximab.
Juvenile pemphigus vulgaris is rare and its occasionally clinically atypical presentation can result in delayed diagnosis and management. Firstline therapy generally comprises systemic corticosteroids. Some cases of recalcitrant pemphigus vulgaris in adults with a refractory course, despite adequate systemic corticosteroids, have been successfully treated with rituximab, as in our case 2. To our knowledge, only two other cases of juvenile pemphigus vulgaris treated with rituximab have been reported.
寻常型天疱疮是一种罕见的自身免疫性水疱病，仅在儿童中罕见出现。
两名分别为4岁和15岁的年轻女孩出现口腔和/或皮肤水疱（病例2）。通过组织学（基底层上棘层松解）和免疫病理学分析（直接和间接免疫荧光，酶联免疫吸附测定法检测抗桥粒芯糖蛋白-3抗体及抗桥粒芯糖蛋白-1抗体阳性或阴性）确诊为寻常型天疱疮。在病例2中，因患者对皮质类固醇产生依赖且静脉注射免疫球蛋白治疗失败，遂使用利妥昔单抗成功治疗。
青少年寻常型天疱疮罕见，其临床表现偶尔不典型，可能导致诊断和治疗延迟。一线治疗通常包括全身使用皮质类固醇。在一些成年寻常型天疱疮患者中，尽管使用了足够的全身皮质类固醇，但病程顽固难治，如我们的病例2，使用利妥昔单抗治疗取得了成功。据我们所知，仅有另外两例青少年寻常型天疱疮使用利妥昔单抗治疗的病例报告。
