Faye-Petersen O, Frankel S R, Schulman P E, Raucher H, Spiera H, Dische M R
Department of Pathology, Mount Sinai Hospital, New York, New York 10029.
Pediatr Pathol. 1991 Mar-Apr;11(2):281-95. doi: 10.3109/15513819109064765.
We describe an 18-year-old white male who developed lower extremity ischemia requiring amputation. He presented at 14 with pulmonary infiltrates, hepatosplenomegaly, fever, rash, adenopathy, uveitis, and arthralgias; clinical and laboratory findings were consistent with Mycoplasma pneumoniae infection. Despite adequate treatment with antibiotics, he developed chronic arthralgias and fevers, with rash and pericardial effusion. Criteria for the diagnosis of systemic lupus erythematosus were not met; juvenile rheumatoid arthritis was diagnosed presumptively. Over the subsequent 4 years he developed lymphadenopathy with biopsy-proven nonnecrotizing granulomas, chronic leg ulceration with granulomatous histology, and acute-onset impending gangrene of the left foot. A biopsy of the posterior tibial artery demonstrated giant cell arteritis. Although the histologic features were consistent with Takayasu's arteritis, complete aortic arteriography was normal. Examination of the amputated leg showed multifocal segmental giant cell arteritis. Clinicopathologic features suggested, but were not fully consistent with, juvenile systemic granulomatosis. His disease may represent a separate sarcoid-like entity in the broad spectrum of vasculitis.
我们描述了一名18岁的白人男性，他因下肢缺血而需要截肢。他14岁时出现肺部浸润、肝脾肿大、发热、皮疹、淋巴结病、葡萄膜炎和关节痛；临床和实验室检查结果与肺炎支原体感染相符。尽管使用抗生素进行了充分治疗，但他仍出现慢性关节痛和发热，并伴有皮疹和心包积液。未满足系统性红斑狼疮的诊断标准；初步诊断为青少年类风湿关节炎。在随后的4年里，他出现了淋巴结病，活检证实为非坏死性肉芽肿，慢性腿部溃疡，组织学表现为肉芽肿，以及左脚急性发作的濒死性坏疽。胫后动脉活检显示巨细胞动脉炎。尽管组织学特征与高安动脉炎一致，但完整的主动脉血管造影正常。对截肢腿部的检查显示多灶性节段性巨细胞动脉炎。临床病理特征提示，但并不完全符合青少年系统性肉芽肿病。他的疾病可能代表了广泛血管炎谱中的一种单独的类肉瘤样实体。
