Eastern Virginia Medical School, Norfolk, VA, USA.
Department of Pediatric Rheumatology, Children's Hospital of The King's Daughters, Eastern Virginia Medical School, 601 Children's Lane, Norfolk, VA, 23507, USA.
Rheumatol Int. 2020 Nov;40(11):1883-1890. doi: 10.1007/s00296-019-04495-2. Epub 2020 Jan 3.
Dermatomyositis (DM) is a multi-system disease that results in chronic inflammation principally of the skin and striated muscle. Small blood vessel injury in the GI tract has been described in dermatomyositis, manifesting as bleeding, ulceration, pneumatosis intestinalis, and ultimately perforation. Recent histopathological studies have shown deposits in the capillaries of the skin, gastrointestinal tract, and brain of patients with dermatomyositis similar to that found in patients with Degos disease, suggesting these disease processes are closely related or represent varying degrees of severity on the same pathologic spectrum. We report a case of juvenile dermatomyositis (JDM) resembling late-stage Degos disease with gastrointestinal perforations successfully treated with combination rituximab and cyclophosphamide therapy. We systematically reviewed the literature detailing the medical and surgical treatments for gastrointestinal perforation in dermatomyositis, Degos-like dermatomyositis, and Degos disease. In addition to our case, as of October 2019, we identified 36 cases describing gastrointestinal perforation in patients with underlying dermatomyositis, 5 cases of Degos-like dermatomyositis and 17 cases of idiopathic Degos disease. Corticosteroid therapy was used widely for dermatomyositis and Degos-like dermatomyositis, while antiplatelet and anticoagulant medications were chiefly used for patients with idiopathic Degos disease. However, there were no cases that detailed the successful treatment of dermatomyositis or Degos disease with gastrointestinal perforation with rituximab alone or combined with cyclophosphamide. We report that rituximab, in combination with cyclophosphamide, can be used as a novel adjunctive therapy to successfully treat dermatomyositis with Degos-like gastrointestinal perforation.
皮肌炎（DM）是一种多系统疾病，主要导致皮肤和横纹肌的慢性炎症。在皮肌炎中已经描述了胃肠道的小血管损伤，表现为出血、溃疡、肠气肿，最终穿孔。最近的组织病理学研究表明，皮肌炎患者的皮肤、胃肠道和大脑毛细血管中有沉积物，类似于 Degos 病患者，这表明这些疾病过程密切相关，或者在同一病理谱上代表不同程度的严重程度。我们报告了一例类似于晚期 Degos 病的青少年皮肌炎（JDM），其胃肠道穿孔成功地接受了利妥昔单抗和环磷酰胺联合治疗。我们系统地回顾了文献，详细描述了皮肌炎、Degos 样皮肌炎和 Degos 病胃肠道穿孔的内科和外科治疗。除了我们的病例，截至 2019 年 10 月，我们共发现 36 例潜在皮肌炎患者发生胃肠道穿孔，5 例 Degos 样皮肌炎和 17 例特发性 Degos 病。皮质类固醇治疗广泛用于皮肌炎和 Degos 样皮肌炎，而抗血小板和抗凝药物主要用于特发性 Degos 病患者。然而，没有病例详细描述利妥昔单抗单独或联合环磷酰胺成功治疗皮肌炎或 Degos 病合并胃肠道穿孔。我们报告利妥昔单抗联合环磷酰胺可作为治疗具有 Degos 样胃肠道穿孔的皮肌炎的一种新的辅助治疗方法。
