临床病例评析——胰腺炎 脂膜炎和关节炎

Pancreatitis, panniculitis, and polyarthritis

目的提高临床对胰腺炎、脂膜炎和关节炎综合征（PPP综合征）的认识。方法描述1例67岁PPP综合征患者的诊治过程，总结其临床、影像学和病理特点。结果患者长期酗酒，临床主要表现为多发皮下结节和多关节肿痛，不伴腹痛。血清胰淀粉酶和脂肪酶显著升高；腹部cT提示慢性胰腺炎；关节MRI提示骨坏死；皮下结节病理可见“鬼影细胞”。糖皮质激素治疗无效，控制胰腺炎后病情逐步缓解。结论PPP综合征腹部症状轻微，易漏诊，应采取针对胰腺疾病的治疗方案。

Objective To improve the recognition of Pancreatitis, Panniculitis, and Polyarthritis syndrome （PPP syndrome）. Methods We described the diagnosis and treatment of a patient with PPP syndrome, including the clinical, radiological, and pathological features. Results Here we report a 67-year-old man with chronic calcified pancreatitis with multiple subcutaneous nodules and polyarthritis, but without any abdominal pain or other abdominal symptoms. His serum pancreatic amylase and lipase were markedly elevated. Abdomen CT scan showed features of chronic calcified panereatitis. MR imaging of ankle revealed intramedullary fat necrosis. Biopsy from subcutaneous swellings revealed fat necrosis without vasculitis and typical ＂ghost-like cells＂. He failed to response to corticosteroids therapy. When the diagnosis of pancreatitis was confirmed, he was managed conservatively with supportive measures, and recovered. Conclusion The diagnosis of PPP syndrome is often difficult when abdominal symptoms are absent, be aware the association between panniculitis and polyarthritis with pancreatic disease may lead to a prompt diagnosis and management.