Pancreatitis-Panniculitis-Polyarthritis-syndrome K85.91

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.

The incidence is 2-3% of all inflammatory or neoplastic pancreatic diseases. In contrast, the complete triad of PPP syndrome occurs much less frequently. Polyarthritis usually manifests symmetrically in the large joints of the extremities. About 2/3 of the patients show no or mild abdominal symptoms, so that a delayed diagnosis and causal therapy have considerable consequences.

The exact pathomechanism is still hypothetical. It can be assumed that an interaction of peripherally active enzymes such as lipases, amylases and trypsins and an activated inflammatory cascade with peripherally active cytokines, free radicals and other vasoactive substances can be held responsible for the tissue destruction. A correlation between the serum amylase concentrationand the progression of adipose tissue necrosis can be assumed.

In 10% of cases, evidence of osteolytic bone lesions, possibly with bone marrow necrosis.

Frequent eosinophilia.

The triad of arthritis, panniculitis of the skin, and eosinophilia is known as the Schmid triad.

Amylase, lipase, and trypsin are elevated due to the diseased pancreas and cause fatty necrosis in subcutaneous adipose tissue, synovium, and bone marrow

Antibiotic treatment and radical surgical debridement of the adipose tissue necrosis guide the final outcome.

The syndrome is more commonly associated with pancreatic cancer than with pancreatitis.

History: A 52-year-old man was admitted with swelling of wrists and ankles. A bland abdomen revealed massively elevated pancreatic enzymes.

Procedure: CT scan showed acute pancreatitis. Within a few days the swelling of the joints increased with necrosis perforation.

Therapy: A necrosectomy, incision and debridement on all 4 limb parts was done. A uniform picture of purulent liquid fat tissue necrosis through all tissue layers down to the bone was seen. Smears remained without evidence of germs.

Course: During the therapy of the pancreatitis the laboratory parameters were regressive. Accompanied by regular necrosectomy, the initially amputation-worthy situation improved in all 4 extremities. Secondary wound healing was achieved at the wrists. At the lower leg tissue defects plastic coverings or partial amputations will be necessary.

Prognosis: The course depends on the osteitis, the extent of the functional deficit of the extremities and last but not least on the course of the underlying disease.

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.