胰腺炎,脂膜炎,和多关节炎(PPP)综合征由于其稀有性和异质性的初始表现,在诊断和管理方面提出了独特的挑战。这份手稿提供了两个PPP综合征患者的病例系列,在诊断过程中发光,并护理这种罕见的情况。PPP综合征的特征是同时发生胰腺炎或假性囊肿以及多关节炎和脂膜炎。虽然其确切的病理生理学仍然晦涩难懂,胰腺炎症被认为会引发胰腺酶的血源性传播,导致脂肪坏死和随后的脂膜炎,以及软骨坏死和/或骨坏死引起的多关节炎。尽管自1980年代后期以来在医学文献中得到认可,PPP综合征仍然知之甚少,全球报告的病例数量有限。它的稀有性和多样的初始表现往往导致误诊,造成适当治疗的延误。所呈现的病例系列突出了PPP综合征的关键临床特征和诊断线索。两名患者均表现出炎性多关节炎的初始症状,伴随着皮肤活检上“鬼细胞”的特征性发现。此外,影像学和实验室证据显示胰腺改变与该综合征一致.该病例系列强调了多学科合作在管理PPP综合征中的重要性。早期识别和准确诊断是启动及时有效的治疗干预措施的关键。从而改善患者的预后并最大限度地减少长期后遗症。
Pancreatitis, panniculitis, and
polyarthritis (PPP) syndrome presents a unique challenge in diagnosis and management because of its rarity and heterogeneous initial presentation. This manuscript presents a case series of two patients with PPP syndrome, shedding light on the diagnostic process and care for this uncommon condition. PPP syndrome is characterized by the simultaneous occurrence of pancreatitis or pseudocysts alongside
polyarthritis and panniculitis. While its exact pathophysiology remains obscure, pancreatic inflammation is assumed to trigger the hematogenous dissemination of pancreatic enzymes, leading to fat necrosis and subsequent panniculitis, as well as chondronecrosis and/or osteonecrosis causing
polyarthritis. Despite its recognition in medical literature since the late 1980s, PPP syndrome remains poorly understood, with only a limited number of cases reported globally. Its rarity and varied initial manifestations often result in misdiagnosis, causing delays in appropriate treatment. The presented case series highlights key clinical features and diagnostic clues of PPP syndrome. Both patients exhibited initial symptoms of inflammatory
polyarthritis, accompanied by characteristic findings of \"ghost cells\" on skin biopsy. Additionally, radiographic and laboratory evidence revealed pancreatic changes consistent with this syndrome. This case series underscores the importance of multidisciplinary collaboration in managing PPP syndrome. Early recognition and accurate diagnosis are pivotal in initiating prompt and effective therapeutic interventions, thereby improving patient outcomes and minimizing long-term sequelae.