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认识PPP综合征:胰腺炎、脂膜炎和多关节炎的罕见组合。

Understanding the PPP Syndrome: A Rare Combination of Pancreatitis, Panniculitis, and Polyarthritis.

作者信息

Bagheri-Ghalehsalimi Amin, Jafari Mohammad, Aria Amir, Kouchak Athar, Momenzadeh Mahnaz

机构信息

Isfahan Gastroenterology and Hepatology Research Center, Isfahan University of Medical Sciences, Isfahan, Iran.

Department of Internal Medicine, Alzahra Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.

出版信息

Adv Biomed Res. 2025 Feb 28;14:14. doi: 10.4103/abr.abr_324_24. eCollection 2025.

DOI:10.4103/abr.abr_324_24
PMID:40213583
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11981042/
Abstract

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but serious disease that can be deadly. Its symptoms come on suddenly and unexpectedly. This disease is identified by erythematous bullous skin lesions and arthritis caused by pancreatic dysfunction. A 25-year-old man had an abdominal trauma with no past medical history. A CT scan showed pancreatic inflammation, peripancreatic edema, and mild fluid in the head of the pancreas without any collection and collection of blood in the retroperitoneum. The patient came back to the hospital 2 weeks later with a fever, abdominal pain, tender joints, redness, warmth in arms and legs, and rash on the skin. In addition, their amylase and lipase levels were increased. Based on the symptoms, the patient was referred to a rheumatologist and diagnosed with PPP syndrome with concomitant pancreatitis, panniculitis, and polyarthritis. Despite being prescribed corticosteroids and broad-spectrum antibiotics, the patient passed away. The exact pathophysiology of the additional abdominal symptoms has not yet been determined. However, some researchers have suggested that this disorder may be due to the entry of pancreatic lipase enzymes into the bloodstream. Pancreatic enzymes damage adipose tissue in the lower extremities and cause panniculitis (subcutaneous adipose tissue inflammation). The symptoms of arthritis may vary depending on the clinical case as different joints are affected and the number and symmetry of joints involved may differ. Therefore, PPP syndrome is sporadic and not easily diagnosed.

摘要

胰腺炎、脂膜炎和多关节炎(PPP)综合征是一种罕见但严重的疾病,可能会致命。其症状会突然且意外地出现。这种疾病通过由胰腺功能障碍引起的红斑性大疱性皮肤病变和关节炎来确诊。一名25岁男性既往无病史,发生了腹部创伤。CT扫描显示胰腺炎症、胰腺周围水肿,胰腺头部有少量液体,后腹膜无任何积液和积血。两周后该患者因发热、腹痛、关节压痛、手臂和腿部发红、发热以及皮肤出现皮疹回到医院。此外,其淀粉酶和脂肪酶水平升高。根据这些症状,该患者被转诊给风湿病专家,并被诊断为伴有胰腺炎、脂膜炎和多关节炎的PPP综合征。尽管使用了皮质类固醇和广谱抗生素治疗,该患者仍去世。额外腹部症状的确切病理生理学尚未确定。然而,一些研究人员认为这种病症可能是由于胰腺脂肪酶进入血液所致。胰腺酶会损害下肢的脂肪组织并导致脂膜炎(皮下脂肪组织炎症)。关节炎的症状可能因临床病例而异,因为不同的关节会受到影响,受累关节的数量和对称性也可能不同。因此,PPP综合征是散发性的,不易诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6312/11981042/c240ccf5f3cf/ABR-14-14-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6312/11981042/99310e2bfbde/ABR-14-14-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6312/11981042/c240ccf5f3cf/ABR-14-14-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6312/11981042/99310e2bfbde/ABR-14-14-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6312/11981042/c240ccf5f3cf/ABR-14-14-g002.jpg

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本文引用的文献

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2
Pancreatitis, Panniculitis, and Polyarthritis (PPP) Syndrome in the Setting of Severe Acute Pancreatitis.重症急性胰腺炎背景下的胰腺炎、脂膜炎和多关节炎(PPP)综合征
Cureus. 2023 Mar 4;15(3):e35768. doi: 10.7759/cureus.35768. eCollection 2023 Mar.
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The Pancreatitis, Panniculitis, and Polyarthritis (PPP) Syndrome: Subcutaneous Nodular Fat Necrosis, a Cutaneous Marker of Pancreatic Neoplasia.
胰腺炎、脂膜炎和多关节炎(PPP)综合征:皮下结节性脂肪坏死,胰腺肿瘤的皮肤标志物。
Acta Dermatovenerol Croat. 2021 Apr;291(1):46-50.
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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。
Clin Rheumatol. 2021 Apr;40(4):1625-1633. doi: 10.1007/s10067-020-05333-8. Epub 2020 Aug 10.
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Scott Med J. 2020 Feb;65(1):19-23. doi: 10.1177/0036933019897373. Epub 2019 Dec 30.
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