Gupta Aman, Pilania Rakesh Kumar, Prasad Venkata Durga, Guleria Sandesh
Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
BMJ Case Rep. 2018 Jan 23;2018:bcr-2017-222915. doi: 10.1136/bcr-2017-222915.
Juvenile dermatomyositis (JDM) is a multisystemic disorder. Vasculitic ulcers in JDM have been reported to involve axilla, elbow or extensor surfaces of other joints. We report a young boy with JDM who presented with extensive cutaneous ulcers involving scrotum, prepuce, gluteal region, neck, bilateral axilla, periumbilical area and bilateral elbows and popliteal fossa. His disease course was marked by several relapses and he required immunosuppression with prednisolone, subcutaneous methotrexate and intravenous cyclophosphamide. His muscle weakness improved and skin ulcers healed after 6 months of intensive immunosuppressive therapy. Children with JDM and ulcers often show increased resistance to immunosuppressive therapy. Extensive cutaneous ulcers in JDM, especially those involving the scrotum, have never been described. Awareness regarding the uncommon manifestations is important to guide appropriate therapy.
幼年皮肌炎（JDM）是一种多系统疾病。据报道，JDM中的血管炎性溃疡累及腋窝、肘部或其他关节的伸侧表面。我们报告了一名患有JDM的小男孩，他出现了广泛的皮肤溃疡，累及阴囊、包皮、臀区、颈部、双侧腋窝、脐周区域以及双侧肘部和腘窝。他的病程以多次复发为特征，需要使用泼尼松龙、皮下注射甲氨蝶呤和静脉注射环磷酰胺进行免疫抑制治疗。经过6个月的强化免疫抑制治疗后，他的肌肉无力症状有所改善，皮肤溃疡也愈合了。患有JDM和溃疡的儿童通常对免疫抑制治疗的耐药性增加。JDM中广泛的皮肤溃疡，尤其是累及阴囊的溃疡，此前从未有过描述。认识到这些不常见的表现对于指导适当的治疗很重要。
