Fatal case of pancreatic panniculitis and polyarthritis due to pancreatic neuroendocrine tumor: a case report and review of literature.

Pancreatic panniculitis (PP) and arthritis may be extrapancreatic manifestations of pancreatic disease. The triad of pancreatic disease, panniculitis and polyarthritis, described in the literature as the PPP syndrome, is sometimes observed in patients with acute or chronic pancreatitis, pancreatic cancer or neuroendocrine tumors (NETs). We present a 60-year-old man with polyarthritis and clinically aggressive PP of the limbs. Computed tomography showed a large tumor in the uncinate process of the pancreas and multiple metastatic lesions in the liver and lungs. The skin nodules developed before the diagnosis of the occult pancreatic tumor and metastases. Biopsy of the skin nodules was performed and showed lobular panniculitis. In addition, immunopathology of the liver metastasis showed NET, a rare pathological variant. The skin lesions immediately spread to the upper limbs with extensive ulceration and necrosis, accompanied by high serum lipase levels. The patient died 4 months after the initial development of the skin lesion. PPP syndrome is a rarely recognized clinical entity. The present case report and literature review highlight the key clinical features of PPP syndrome. The absence of general or abdominal symptoms and skin changes mimicking other forms of panniculitis may lead to delayed diagnosis. It is important to broaden the diagnostic work-up for pancreatic disease in patients presenting with panniculitis and arthritis.