Jira M, Elqatni M, Sekkach Y, Elomri N, Mekouar F, Ghafir D
Service de médecine interne B, hôpital militaire d'instruction Mohammed V, avenue des Far, Hay Riad, Rabat, Maroc.
Ann Dermatol Venereol. 2013 Dec;140(12):778-83. doi: 10.1016/j.annder.2013.04.087. Epub 2013 May 27.
Bullous lupus is a subepidermal autoimmune bullous dermatosis, a rare entity that forms one of the cutaneous signs of systemic lupus erythematosus. We report on the clinical, immunopathological and progressive features of bullous lupus in three patients.
Our patients consisted of two women and one man aged 34, 22 and 30 years respectively. A diagnosis of bullous lupus erythematosus was evoked by blisters or vesicular blisters and confirmed, in addition to criteria for the diagnosis of systemic lupus erythematosus, by the presence of subepidermal blistering with infiltrate containing neutrophils and eosinophils as revealed by histological analysis, and of deposits of IgG and IgM (two cases) or of IgA (one case) at the dermo-epidermal junction observed under direct immunofluorescence. Indirect immunofluorescence showed anti-collagen VII antibodies. Lupus nephritis was present in two cases. Our patients were treated with corticosteroids and immunosuppressants.
Bullous lupus erythematosus may be the first sign of systemic lupus erythematosus with severe visceral involvement, especially renal involvement, suggesting that it may be a marker of activity and prognosis.
大疱性狼疮是一种表皮下自身免疫性大疱性皮肤病，是一种罕见的疾病，是系统性红斑狼疮的皮肤表现之一。我们报告了3例大疱性狼疮的临床、免疫病理及进展特征。
我们的患者包括2名女性和1名男性，年龄分别为34岁、22岁和30岁。大疱性红斑狼疮的诊断是由水疱或水疱性水疱引发的，除了系统性红斑狼疮的诊断标准外，组织学分析显示表皮下水疱形成，伴有含中性粒细胞和嗜酸性粒细胞的浸润，直接免疫荧光观察显示在真皮-表皮交界处有IgG和IgM沉积（2例）或IgA沉积（1例），从而得以确诊。间接免疫荧光显示抗VII型胶原抗体。2例患者存在狼疮性肾炎。我们的患者接受了糖皮质激素和免疫抑制剂治疗。
大疱性红斑狼疮可能是系统性红斑狼疮伴有严重内脏受累尤其是肾脏受累的首发症状，提示它可能是疾病活动度和预后的一个指标。
