ObjectiveTo investigate the clinical characteristics of pancreatitis, panniculitis, and polyarthritis syndrome (PPP syndrome). MethodsA patient with PPP syndrome in July 2013 was reported. The clinical features of 33 cases of PPP syndrome at home and abroad were reviewed. ResultsOur patient with panniculitis and arthritis as the first manifestation had few abdominal symptoms despite high serum levels of pancreatic enzymes. In the 34 PPP cases, 70.6% (24/34) had absent or mild abdominal symptoms, easily leading to misdiagnosis. Multi-joint involvement was more common, and in almost 1/3 of the patients, joint symptoms with poor NSAIDs and/or hormonal responses predated the identification of pancreatic disease. Panniculitis biopsy found characteristic "ghost cells". Ten patients died of complications of pancreatic disease (including 2 cases of cancer), and the mortality rate was as high as 29.4% (10/34). ConclusionPPP syndrome with mild abdominal symptoms and high mortality should be taken for the treatment of pancreatic disease, to prevent misdiagnosis and mistreatment.