Pancreatitis, polyarthritis and panniculitis syndrome

The pancreatitis-panniculitis-polyarthritis syndrome (PPP syndrome) is a clinical symptom complex that occurs in patients with pancreatitis (or an acinar-endocrine carcinoma of the pancreas), with (pancreatic) aseptic panniculitis and polyarthritis (sometimes with osteonecrosis of varying severity). The cause lies in the (massive) release of pancreatic enzymes such as trypsin, lipase and amylase, which leads to aseptic necrosis in the fatty tissue far from the heart.

1. Azar L et al. (2014) Pancreatitis, polyarthritis and panniculitis syndrome. Joint Bone Spine revue du rhumatisme 81:184.
2. Chiari H. On the so-called fat necrosis. Prag. Med. weekly 8:255-256.
3. Dieker W et al.(2017) 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. Int J Surg Case Rep 31: 170-175.
4. Fraisse T et al. (2010) Pancreatitis, panniculitis, polyarthritis syndrome: an unusual cause of destructive polyarthritis. Joint Bone Spine revue du rhumatisme 77:617-618.
5. Kim EJ et al.(2019) Pancreatitis, panniculitis, and polyarthritis syndrome simulating cellulitis and gouty arthritis. Korean J Gastroenterol 74:175-182.
6. Kuwatani M et al. (2010) Osteonecrosis and panniculitis as life-threatening signs. Clin. Gastroenterol. Hepatol 8:e52-53.
7. Laureano A et al. (2014) Pancreatic panniculitis - a cutaneous manifestation of acute pancreatitis. J Dermatol. Case Rep 8:35-37.
8. Narvaez J et al (2010) Pancreatitis, panniculitis, and polyarthritis. Semin. Arthritis Rheum. 39:417-423.