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。胃肠道中的小血管损伤已被描述为皮肌炎，表现为出血、溃疡、肠积气，并最终穿孔。最近的组织病理学研究表明，皮肌炎患者的皮肤、胃肠道和大脑的毛细血管沉积物与德戈斯病患者的沉积物相似，表明这些疾病过程密切相关或在同一病理谱上表现出不同程度的严重程度. 我们报告了一例类似晚期德戈斯病伴胃肠道穿孔的青少年皮肌炎 (JDM)，利妥昔单抗和环磷酰胺联合治疗成功。我们系统地回顾了详细介绍皮肌炎、德戈斯样皮肌炎和德戈斯病胃肠穿孔的内科和外科治疗的文献。除我们的病例外，截至 2019 年 10 月，我们还确定了 36 例描述潜在皮肌炎患者胃肠道穿孔的病例、5 例德戈斯样皮肌炎和 17 例特发性德戈斯病。皮质类固醇治疗广泛用于皮肌炎和德戈斯样皮肌炎，而抗血小板和抗凝药物主要用于特发性德戈斯病患者。然而，尚无单独使用利妥昔单抗或联合环磷酰胺成功治疗皮肌炎或德戈斯病伴胃肠穿孔的病例。我们报告利妥昔单抗，