Published online Sep 26, 2019. doi: 10.12998/wjcc.v7.i18.2808
Peer-review started: April 23, 2019
First decision: August 1, 2019
Revised: August 17, 2019
Accepted: August 27, 2019
Article in press: August 26, 2019
Published online: September 26, 2019
Processing time: 158 Days and 1.7 Hours
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.
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.
PPP syndrome should be suspected when accompanied by skin and joint lesions. Delays in diagnosis could have catastrophic consequences.
Core tip: Panniculitis, polyarthritis, and pancreatitis syndrome must be considered when skin lesions and joint symptoms are associated with suspected pancreatitis. Treatment of the causative pancreatic disease is helpful in relieving symptoms. Surgical treatment is needed when fistulas of the pancreatic duct and systemic circulation formed. Delays in diagnosis could have catastrophic consequences.