Ito Yumi, Takeuchi Saki, Tozawa Takahisa, Hisada Satoko, Yamada Yoshihiro, Kodera Masanari, Kobayashi Masahiro, Shirahata Mizuho, Matsubara Akihiro
Department of Dermatology, Japan Community Health Care Organization Chukyo Hospital, Nagoya, Japan.
Department of Respiratory Medicine, Japan Community Health Care Organization Chukyo Hospital, Nagoya, Japan.
J Dermatol. 2024 Mar;51(3):448-452. doi: 10.1111/1346-8138.17004. Epub 2023 Oct 13.
This case study illustrates a 63-year-old Japanese woman who presented with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis. She was administered a therapeutic regimen consisting of corticosteroids, tacrolimus, and cyclophosphamide. However, after a month of treatment, symptoms of confusion and depressive tendencies emerged, followed by the manifestation of hematuria, thrombocytopenia, and fragmented erythrocytes. A disintegrin-like and metalloprotease with thrombospondin type 1 motifs 13 activity was 45%. Thrombotic microangiopathy was contemplated, yet a definitive diagnosis remained elusive. She died 2 months after admission. Although the occurrence of thrombotic microangiopathy in patients with dermatomyositis is rare, the prognosis is poor, emphasizing the importance of prompt diagnosis and treatment.
本病例研究展示了一名63岁的日本女性，她患有抗黑色素瘤分化相关基因5抗体阳性皮肌炎。她接受了由皮质类固醇、他克莫司和环磷酰胺组成的治疗方案。然而，治疗一个月后，出现了意识模糊和抑郁倾向的症状，随后出现血尿、血小板减少和破碎红细胞。具有血小板反应蛋白1型基序的去整合素样金属蛋白酶13活性为45%。考虑为血栓性微血管病，但仍难以明确诊断。她入院2个月后死亡。虽然皮肌炎患者发生血栓性微血管病的情况罕见，但预后较差，这强调了及时诊断和治疗的重要性。
