Pancreatitis, Panniculitis, Polyarthritis Syndrome (PPP syndrome): A Case Report
Abstract
Pancreatitis presenting without abdominal pain is very unusual. A 35-year-old man with acute pancreatitis presented with prolonged fever, polyarthritis and multiple subcutaneous swellings, but without any abdominal pain. His serum lipase and amylase were very high. Biopsy from the subcutaneous swellings revealed fat necrosis and CT scan abdomen showed features of acute pancreatitis. He was managed conservatively. The available literature on this syndrome was reviewed.
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Introduction
The pancreatic disease, panniculitis, polyarthritis (PPP) syndrome was first described by Berner in 19081 . Although the pancreatic pathology with high blood levels of pancreatic enzymes is considered causative, abdominal symptoms are often mild or absent. The delay in diagnosis and specific treatment of the underlying pancreatitis worsens the prognosis of this condition, which has a high mortality rate2 . In nearly 45% of the patients, the arthritis follows a chronic course with a poor response to nonsteroidal anti-inflammatory drugs and corticosteroids, and the rapid development of radiographic joint damage.
As this is rare cases so the case was thoroughly evaluated and a case report was prepared to publish.
Conclusion
‘PPP’ syndrome is a very rare clinical entity. Only about 66 cases have been reported till date. It is often misdiagnosed. Pancreatitis in these patients is usually silent with no clinical symptoms pertaining to the pathology and hence patient is diagnosed only late in the course. Histopathological demonstration of panniculitis is a must for diagnosis. Management is nonspecific and no specific treatment is available presently .Patients are treated for pancreatitis and often require long term NSAIDs for prolonged fever and Total pancreatectomy is indicated in cases of pancreatic carcinoma.