Symptoms of PPP syndrome
What are the clinical features of the pancreatic, panniculitis, and polyarthritis (PPP) syndrome?
PPP is a systemic syndrome occurring in some patients with pancreatitis or pancreatic acinar cell carcinoma due to release of trypsin, lipase, and amylase from the diseased pancreas causing fat necrosis. A good way to remember the clinical manifestations is the mnemonic PANCREAS:
P — P ancreatitis
A — A rthritis (60%) and arthralgias, usually of the ankles and knees. Synovial fluid is typically noninflammatory and creamy in color due to lipid droplets that stain with Sudan black or oil red O.
N — N odules that are tender, red, and usually on the extremities. These are frequently misdiagnosed as erythema nodosum but are areas of lobular (not septal) panniculitis with fat necrosis. Fasciitis due to subcutaneous fat necrosis can also be seen.
C — C ancer of the pancreas more commonly causes this syndrome than does pancreatitis.
R — R adiographic abnormalities due to osteolytic bone lesions from bone marrow necrosis (10%).
E — E osinophilia. The triad of arthritis, nodules, and eosinophilia is called Schmidt’s triad.
A — A mylase, lipase, and trypsin are elevated due to release by a diseased pancreas and cause the fat necrosis in skin, synovium, and bone marrow.
S — S erositis, including pleuropericarditis frequently with fever.