Pancreatitis, panniculitis and polyarthritis (PPP-) syndrome caused by post-pancreatitis pseudocyst with mesenteric fistula. Diagnosis and successful surgical treatment. Case report and review of literature. (2017)

• Record Type: Journal Article
• Authors: Dieker, Wulf; Derer, Johannes; Henzler, Thomas; Schneider, Alexander; Rückert, Felix; Wilhelm, Torsten J.; Krüger, Bernd
• Is Part Of: International journal of surgery case reports. Volume 31(2017)
• Page Start: 170
• Page End: 175
• Publication Date: 2017
• Subjects: Pancreatitis -- Arthritis -- Fat tissue necrosis -- Pancreatic pseudocyst -- Lipase -- Osteonecrosis; Surgery -- Periodicals; Surgical Procedures, Operative -- Periodicals; Surgery; Electronic journals; Periodicals; 617.005
• DOI: 10.1016/j.ijscr.2017.01.037
• Languages: English
• ISSNs: 2210-2612

Abstract

Highlights: Pancreatitis, panniculitis and polyarthritis syndrome is a very rare manifestation of pancreatitis with panniculitis and polyarthritis with intraosseous fat necrosis. A rare differential diagnosis of unclear polyarthritis, panniculitis or osteonecrosis. Surgical treatment, if possible can lead to complete remission. Abstract: Introduction: Pancreatitis, panniculitis and polyarthritis syndrome is a very rare extra-pancreatic complication of pancreatic diseases. Presentation of case: While in most cases this syndrome is caused by acute or chronic pancreatitis, we report a case of a 62-year-old man presenting with extensive intraosseous fat necrosis, polyarthritis and panniculitis caused by a post-pancreatitis pseudocyst with a fistula to the superior mesenteric vein and extremely high blood levels of lipase. This became symptomatic 2.5 years after an episode of acute pancreatitis and as in most cases abdominal symptoms were absent. Treatment by surgical resection of the pancreatic head with the pseudocyst and mesenteric fistula led to complete remission of all symptoms. Discussion: A review of the literature revealed that all publications are limited to case reports. Most authors hypothesize that an unspecific damage can cause a secretion of pancreatic enzymes to the bloodstream leading to a systemic lipolysis and fat tissue necrosis, especially of subcutaneous tissue, bone marrow, inducing panniculitis, polyarthritis and osteonecrosis. Even if caused by an acute pancreatitis abdominal symptoms are often mild or absent in most cases leading to misdiagnosis and poor prognosis. Conclusion: While symptomatic treatment with NSAR and cortisone showed poor to moderate response, causal treatment can be successful depending on the underlying pancreatic disease.