Premature diagnosis of calciphylaxis without pathological indications finally diagnosed as cutaneous small-vessel vasculitis: a post-mortem case report.

Nihon Jinzo Gakkai shi Pub Date : 2017-01-01
Hironori Nakamura, Mariko Anayama, Yasushi Makino, Masaki Nagasawa
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Abstract

A man in his fifties with diabetes had a past history of myocardial infarction and ventricular septal perforation. He underwent hemodialysis about a year ago and was taking amiodarone. He presented with sores and purpura on the lower limbs.-Skin biopsy showed immunofluorescence-negative leukocytoclastic vasculitis. Skin lesions were treated with ointments, which ameliorated the symptoms to some extent, but ulceration relapsed and deteriorated in both number and size. Calciphylaxis was suspected, and a second skin biopsy was performed. No calcium detection,on the arteries was observed, but leukocytoclastic vasculitis was seen. Antineutrophil cytoplasmic antibody-related vasculitis, cryoglobulin vasculitis, or anti-phospholipid syndrome were ruled out by negative findings for autoantibodies. Although he was treated with 30 mg prednisolone, his systemic condition deteriorated, and he died of disseminated intravascular coagulation. Autopsy findings showed no vasculitis in the lung, kidney or intestine, and perimyocardial patch infection was observed.Although calciphylaxis was clinically suspected, his condition was diagnosed finally as cutaneous small-vessel vasculitis.

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过早诊断无病理指征的钙化反应最终诊断为皮肤小血管炎:一个死后病例报告。
50多岁糖尿病患者,既往有心肌梗死、室间隔穿孔病史。大约一年前,他接受了血液透析,并服用胺碘酮。他表现出下肢溃疡和紫癜。皮肤活检显示免疫荧光阴性白细胞破裂性血管炎。皮肤病变用软膏治疗,症状得到一定程度的改善,但溃疡复发,溃疡数量和大小均恶化。怀疑有钙化反应,并进行了第二次皮肤活检。动脉未见钙检测,但可见白细胞破裂性血管炎。抗中性粒细胞细胞质抗体相关血管炎、冷球蛋白血管炎或抗磷脂综合征被自身抗体阴性结果排除。尽管他接受了30毫克强的松龙治疗,但他的全身状况恶化,死于弥漫性血管内凝血。尸检结果显示肺、肾、肠均无血管炎,心包贴片感染。虽然临床怀疑有钙化反应,但最终诊断为皮肤小血管炎。
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