Juvenile dermatomyositis resembling late-stage Degos disease with gastrointestinal perforations successfully treated wit

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Juvenile dermatomyositis resembling late‑stage Degos disease with gastrointestinal perforations successfully treated with combination of cyclophosphamide and rituximab: case‑based review William Day1   · Christos Gabriel2   · Robert E. Kelly Jr.3   · Cynthia M. Magro4   · Judith V. Williams5   · Alice Werner6   · Laura Gifford5   · Sameer P. Lapsia7   · Cassyanne L. Aguiar2  Received: 18 June 2019 / Accepted: 10 December 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract 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. Keywords  Dermatomyositis · Degos disease · Rituximab · Cyclophosphamide · Gastrointestinal perforation

Introduction Dermatomyositis is a multi-system disease that results in chronic inflammation principally of the skin and striated muscle [1]. Gastrointestinal (GI) manifestations in dermatomyositis can range from dysphagia due to proximal esophageal muscle dysfunction to dysmotility and delayed gastric emptying from smooth muscle involvement. Small blood vessel injury in the GI tract has also been described in dermatomyositis, manifesting as bleeding, ulceration, pneumatosis intestinalis, and ultimately perforation [1, 2]. * Cassyanne L. Aguiar Cassyanne.Aguiar@chkd