Pancreatitis, panniculitis, and polyarthritis / 中华风湿病学杂志

ABSTRACT

Objective To improve the recognition of Pancreatitis, Panniculitis, and Polyarthritis syndrome (PPP syndrome). Methods We described the diagnosis and treatment of a patient with PPP syndrome, including the clinical, radiological, and pathological features. Results Here we report a 67-year-old man with chronic calcified pancreatitis with multiple subcutaneous nodules and polyarthritis, but without any abdominal pain or other abdominal symptoms. His serum pancreatic amylase and lipase were markedly elevated. Abdomen CT scan showed features of chronic calcified pancreatitis. MR imaging of ankle revealed intramedullary fat necrosis. Biopsy from subcutaneous swellings revealed fat necrosis without vasculitis and typical "ghost-like cells". He failed to response to corticosteroids therapy. When the diagnosis of pancreatitis was confirmed, he was managed conservatively with supportive measures, and recovered. Conclusion The diagnosis of PPP syndrome is often difficult when abdominal symptoms are absent, be aware the association between panniculitis and polyarthritis with pancreatic disease may lead to a prompt diagnosis and management.