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

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.

Keywords: Arthritis; Fat tissue necrosis; Lipase; Osteonecrosis; Pancreatic pseudocyst; Pancreatitis.

Fig. 1

Development of lipase levels. Normal: 73–393 U/l. Treatment for reactive arthritis was 20 mg prednisolone + doxycycline. Operation: Surgical resection of pancreatic pseudocyst.

Fig. 2

2-phase bone scintigraphy (late phase). Symmetric polyarthritis of the extremity skeleton, but atypical intense uptake in the left proximal tibia (arrow).

Fig. 3

MR-Imaging of knee and ankles demonstrated multiple ‘infarct like’ bone necrosis near joints of prox. and distal tibia, calcaneus, talus and all tarsal bones. Diffuse contrast agent uptake of surrounding soft tissue.

Fig. 4

Abdominal MRI (A, B) depicted a pseudocyst of the uncinate process of the pancreas. The cystic lesion shows a direct connection to the superior mesenteric vein on the contrast enhanced T1 weighted images (A) as well as on the T2 weighted images (B) (arrow in A and B). The connection between the cyst and the superior mesenteric vein was also detectable on a contrast enhanced CT study which was performed one week prior to the MRI examination.

Fig. 5

Operative findings showed the opened pseudocyst of the uncinated process (arrow) close to the superior mesenteric vein (SMV). ▼marks the altered segment of SMV with thrombosis. PH = pancreatic head.