Abstract
BACKGROUND: Panniculitis, polyarthritis, and pancreatitis (PPP) syndrome is a triad comprising an extremely rare extra-pancreatic complication of pancreatic disease. Herein, we describe a patient with PPP syndrome caused by fistula formation between the inferior vena cava (IVC) and pancreatic pseudocyst. CASE SUMMARY: A 64-year-old man visited the hospital with bilateral leg pain that began one week prior. He had no specific diseases, except hypertension. His vital signs were normal. Blood test revealed the following findings: White blood cell count, 28690/µL; amylase level, 9055 U/L; lipase level, 2089 U/L; and C-reactive protein level, 12.94 mg/dL. Computed tomography of the pancreas revealed recent acute pancreatitis. Nonsteroidal anti-inflammatory drugs were administered with no improvement. After steroid administration, pain slightly improved. Skin lesions were diagnosed as panniculitis. Bone scan and knee magnetic resonance imaging revealed osteoarthritis and bone marrow infarctions. Surgical treatment was considered; total pancreatectomy with splenectomy was performed. A pseudocyst was present posterior to the head of the pancreas, forming a fistula with the suprarenal IVC. After surgery, amylase and lipase levels decreased. However, the patient died of an uncontrolled infection on the 13th postoperative day. CONCLUSION: PPP syndrome should be suspected when accompanied by skin and joint lesions. Delays in diagnosis could have catastrophic consequences.