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罕见的多关节炎性脂膜炎和胰腺炎病例。

A rare case of polyarthritis panniculitis and pancreatitis.

机构信息

Department of General Internal Medicine, Internal Medicine Residency Program, Lahey Hospital and Medical Center, 41 Burlington Mall Road, Burlington, MA, 01805, USA.

Department of Gastroenterology, Lahey Hospital and Medical Center, 41 Burlington Mall Road, Burlington, MA, 01805, USA.

出版信息

Clin J Gastroenterol. 2024 Aug;17(4):765-770. doi: 10.1007/s12328-024-01977-0. Epub 2024 May 22.

DOI:10.1007/s12328-024-01977-0
PMID:38775908
Abstract

Extra pancreatic manifestations of pancreatitis are rare, with a prevalence of 2-3%. One such rare manifestation is the triad of joint pain (polyarthritis), tender skin lesions (panniculitis), and pancreatic inflammation (pancreatitis), known as PPP. The pathogenesis of this phenomenon is not fully understood but is believed to involve lipolysis by pancreatic enzymes at lipid-rich skin and joint sites. PPP primarily affects middle-aged males with a history of alcohol use disorder. Diagnosis can be challenging due to the absence of typical abdominal symptoms. Delayed diagnosis may significantly worsen outcomes. Supportive therapy is the mainstay, but resolution requires addressing the underlying pancreatic abnormality. We present a case of a patient with a history of alcohol use disorder and recurrent acute pancreatitis who developed joint pain and skin rash. Extensive work-up ruled out other causes, and imaging and biopsy confirmed the diagnosis of PPP. Symptomatic management and treatment of the underlying pancreatic abnormality led to complete resolution of symptoms. Our case serves to raise awareness of this rare but potentially fatal syndrome.

摘要

胰腺炎的胰腺外表现较为罕见,其患病率为 2-3%。这种罕见表现之一是三联征,即关节痛(多发性关节炎)、皮肤触痛性病变(胰腺炎)和胰腺炎(胰腺炎),称为 PPP。这种现象的发病机制尚未完全阐明,但据信涉及胰腺酶在富含脂质的皮肤和关节部位的脂肪分解。PPP 主要影响有酒精使用障碍史的中年男性。由于缺乏典型的腹部症状,诊断具有挑战性。延迟诊断可能会显著恶化预后。支持性治疗是主要治疗方法,但要解决根本的胰腺异常才能得到缓解。我们报告了一例有酒精使用障碍和复发性急性胰腺炎病史的患者,其出现关节痛和皮疹。广泛的检查排除了其他原因,影像学和活检证实了 PPP 的诊断。症状性管理和治疗基础胰腺异常导致症状完全缓解。我们的病例旨在提高对这种罕见但可能致命综合征的认识。

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Clinical characteristics, treatment, and outcome of pancreatitis, panniculitis, and polyarthritis syndrome: a case-based review.胰腺炎、脂膜炎和多发性关节炎综合征的临床特征、治疗和转归:基于病例的综述。
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