S3557 Pancreatitis, Panniculitis, and Polyarthritis: A Case Report

Introduction: Pancreatitis, polyarthritis, and panniculitis (PPP) syndrome is characterized by acute on chronic pancreatitis, mono- or polyarthritis, and panniculitis. Diagnosis can be made by skin biopsy indicating pancreatic panniculitis with ghost cells and saponification. Although described as a clinical triad, patients may not display all 3 characteristics simultaneously, making identifying this syndrome challenging. Research describing the diagnosis, pathophysiology, and management of PPP syndrome remains largely limited to case studies. Case Description/Methods: A 31-year-old man with a medical history notable for acute pancreatitis secondary to alcohol use with subsequent portal vein thrombosis, infected hemorrhagic necrosis, and a pancreatic pseudocyst presented to the emergency department with epigastric pain and worsening diffuse, asymmetric joint pain. Prior to admission, he completed a 5-day course of prednisone for recurrent joint pain with no improvement. Workup revealed elevated lipase, confirming pancreatitis. Computed tomography of the abdomen and pelvis showed a 12 cm × 6 cm pseudocyst and thrombosis at the confluence of the splenic vein and superior mesenteric vein consistent with previous computed tomography. Throughout his hospital course, the patient described worsening joint pain. Similarly, he recalled his prior occurrence of joint pain coinciding with the initial episode of pancreatitis 1 month prior. Throughout hospitalization, physical exam of joints revealed tenderness and swelling of affected joints. On the day of discharge, exam of the lateral right foot showed firmness, violaceous discoloration. A tissue biopsy of this area confirmed pancreatic panniculitis. Discussion: This case report highlights the heterogeneity possible within PPP Syndrome in an attempt to encourage clinicians to consider PPP Syndrome even in the absence of a complete triad. Diagnosis of PPP Syndrome in this patient was dependent on patient’s recall of previous coinciding symptoms and the development of panniculitis during the hospital course. While this syndrome remains understudied, PPP syndrome is associated with significant morbidity and mortality, making early diagnosis via skin biopsy and mitigation of exacerbating factors crucial.