A case report of pancreatitis-panniculitis-polyarthritis syndrome – an unusual but serious presentation of pancreatic disease

Abstract

The clinical triad of peripheral cutaneous erythematous nodules, oligo or polyarthritis with intraosseous fat necrosis in the setting of pancreatic disease defines a rare entity called pancreatitis-panniculitis-polyarthritis syndrome. The early recognition of this triad is critical due to its high mortality rate and the rapid onset of osseous and articular disabilities. We describe the clinical course of a 54-year-old patient with complaints of weight loss and fever who presented to our hospital with signs of polyarthritis and appendicular erythematous cutaneous nodules. Clinical investigation revealed high inflammatory and pancreatic enzymes levels. Cutaneous biopsy and articular MRI showed evidence of peripheral necrosis. After a thorough investigation, a diagnosis of panniculitis-polyarthritis-pancreatitis syndrome was established. Treatment was initiated, and a slow but steady improvement was observed. Further complications of the disease process were observed. This case highlights the importance of recognising the association between panniculitis and polyarthritis with pancreatic disease in order to improve outcomes.

Subcutaneous and Intraosseous Fat Necrosis Associated with Chronic Pancreatitis

“…The clinical symptoms include a febrile state (or not), pain in multiple joints, swelling, and erythema in the affected areas. This syndrome is known as PPP syndrome (pancreatic disease, panniculitis and polyarthritis) [20] and was first described in 1908 by Berner [26]. A 2017 study by Dieker et al reported another 32 cases of this syndrome described in the literature in addition to their case report [25].…”

“…It is generally accepted theory that direct injury to the blood vessels by pancreatic enzymes and excessive release of circulating lipases in the course of pancreatitis results in fat necrosis and intramedullary lipolysis. Some researchers consider that inflammatory response and necrosis in articular areas are caused by free fatty acids which are formed by the hydrolysis of triglycerides under the action of pancreatic lipases [26]. Intra-abdominal fat necrosis of the retroperitoneum, mesentery, and omentum is a frequent complication of acute, chronic, and traumatic pancreatitis [1].…”