Clinical characteristics, treatment, and outcome of pancreatitis, panniculitis, and polyarthritis syndrome: a case-based review

A 42-year-old Caucasian female presented with lower limb panniculitis and bilateral ankle arthritis in the absence of abdominal or other localizing symptoms. Abdominal imaging revealed subacute pancreatitis with pseudocyst formation. The clinical manifestations were compatible with pancreatitis, panniculitis, and polyarthritis syndrome (PPP syndrome), a very rare complication of pancreatic disease. The patient improved with conservative treatment for the pancreatic disease and systemic corticosteroids for the cutaneous and articular manifestations. We identified 59 patients with the PPP syndrome from the literature, the majority of patients being male (74.6%) with a median age of 49 (IQR 41–63.5) years. Acute pancreatitis is the most frequent underlying disorder (54.2%), but gastrointestinal symptoms are absent in 45.8% of patients. Pancreatic panniculitis has a predilection for the lower limbs, which are affected in 98.3% of cases. However, the cutaneous lesions may also involve the upper limbs and trunk. Arthritis is typically symmetric and polyarticular in nature, affecting both large and small joints. Of all patients who received treatment, 78.6% had a poor response. Death occurred in 27.1% of cases after a median duration of 8 (IQR 3.5–14) weeks