Understanding the PPP Syndrome: A Rare Combination of Pancreatitis, Panniculitis, and Polyarthritis

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but serious disease that can be deadly. Its symptoms come on suddenly and unexpectedly. This disease is identified by erythematous bullous skin lesions and arthritis caused by pancreatic dysfunction. A 25-year-old man had an abdominal trauma with no past medical history. A CT scan showed pancreatic inflammation, peripancreatic edema, and mild fluid in the head of the pancreas without any collection and collection of blood in the retroperitoneum. The patient came back to the hospital 2 weeks later with a fever, abdominal pain, tender joints, redness, warmth in arms and legs, and rash on the skin. In addition, their amylase and lipase levels were increased. Based on the symptoms, the patient was referred to a rheumatologist and diagnosed with PPP syndrome with concomitant pancreatitis, panniculitis, and polyarthritis. Despite being prescribed corticosteroids and broad-spectrum antibiotics, the patient passed away. The exact pathophysiology of the additional abdominal symptoms has not yet been determined. However, some researchers have suggested that this disorder may be due to the entry of pancreatic lipase enzymes into the bloodstream. Pancreatic enzymes damage adipose tissue in the lower extremities and cause panniculitis (subcutaneous adipose tissue inflammation). The symptoms of arthritis may vary depending on the clinical case as different joints are affected and the number and symmetry of joints involved may differ. Therefore, PPP syndrome is sporadic and not easily diagnosed.