Abstract
A young male presented with intermittent high-grade fever, asymmetric polyarthritis and erythematous, tender nodules over left shin for 2 months duration. He had a history of alcohol dependence with multiple episodes of acute pancreatitis. With polyarthritis progressing relentlessly, unresponsive to non-steroidal anti-inflammatory drugs and steroids, a provisional diagnosis of sarcoidosis was considered. Indeed, he was treated with azathioprine and rituximab with no effect. Biopsy of the skin nodule revealed subcutaneous fat necrosis, foam cells, deposition of eosinophilic amorphous material and calcification. Synovial fluid aspiration from the arthritic knee obtained purulent but surprisingly culture-negative material, rich in triglycerides. Abdominal CT confirmed chronic pancreatitis. Final diagnosis of pancreatitis, panniculitis and polyarthritis (PPP) syndrome was made. He underwent surgical pancreatic ductal drainage leading to complete remission of symptoms. PPP syndrome triad occurs due to leakage of pancreatic enzymes into systemic circulation and subsequent fat necrosis. Surgical drainage of pancreatic duct is often curative.
Reference8 articles.
1. Zundler S , Erber R , Agaimy A , et al . Pancreatic panniculitis in a patient with pancreatic-type acinar cell carcinoma of the liver--case report and review of literature. BMC Cancer 2016;16:130. doi:10.1186/s12885-016-2184-6
2. 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;Dieker;Int J Surg Case Rep,2017
3. Polyarthritis with chondronecrosis associated with osteonecrosis, panniculitis and pancreatitis;Mustafa;Rheumatol Int,2010
4. Pancreatic panniculitis;Rongioletti;G Ital Dermatol Venereol,2013
5. Pancreatitis, Panniculitis, and polyarthritis syndrome simulating cellulitis and gouty arthritis;Kim;Korean J Gastroenterol,2019