Case Presentation: A 31-year-old man with a history of alcohol use disorder (AUD) and recent pancreatitis presented with epigastric pain, nausea, vomiting and migratory joint pain. His initial pancreatitis episode was about 12 weeks prior, with CT and MRCP demonstrating necrotizing pancreatitis. He improved with supportive care but returned 30 days later with acute elbow swelling and pain. Surgical washout and synovial fluid analysis demonstrated < 3000 WBC/hpf and no crystals. ESR/CRP were elevated but blood and joint cultures had no growth. ANA, rheumatoid factor, anti-CCP, were negative. He was discharged with antibiotics, prednisone, and plan for outpatient orthopedic and rheumatology follow up. He returned 40 days later with epigastric pain, vomiting, continued joint pain and swelling, and skin lesions on his right foot. Exam demonstrated swelling, redness and tenderness of bilateral MCP joints and L wrist (Fig. 1) and erythematous skin lesions R foot (Fig 2). Lipase was >15,000 U/L and a CT scan demonstrated pancreatic pseudocyst. A skin biopsy was obtained from a lesion on his right foot. He improved with cyst gastrostomy and supportive care and was discharged home awaiting rheumatology follow up. His skin biopsy was subsequently read as “subcutaneous panniculitis with neutrophils, basophilic debris/saponification and ghost cells” yielding a diagnosis of pancreatitis, panniculitis, and polyarthritis (PPP) syndrome.
Discussion: Pancreatitis is among the most common gastrointestinal reasons for hospital care in the USA.1 Hospitalists should be familiar with extra-pancreatic manifestations of pancreatitis, including fatigue, chills and arthralgias,2 and dermatologic findings in 2-3% of patients, such as livido reticularis, acanthosis nigricans, and panniculitis.3 Panniculitis is caused by subcutaneous fat necrosis, resulting in erythematous nodules. Panniculitis has a male predominance and is associated with AUDs. Very rarely, patients may also have intraosseous fat necrosis, resulting in an inflammatory polyarthritis, and thus PPP syndrome, as in our case. The etiology of PPP syndrome remains unclear, but is believed to be related to serum lipase extravasating into the peripheral tissues, causing lipolysis and inflammation.4 Diagnosis is established through clinical features and confirmed with skin biopsy, which typically reveals liquefactive fat necrosis. Prompt recognition is imperative as mortality of PPP is estimated at 24%.5 Treatment is supportive, and includes treatment of underlying pancreatitis, and occasionally use of NSAIDs or systemic corticosteroids.6 PPP treatment may require specialties across multiple disciplines, including gastroenterology, rheumatology, and dermatology.
Conclusions: PPP is a rare syndrome that poses risk of significant morbidity and mortality and may utilize extensive health resources. It is essential that PPP is recognized early, as prompt treatment of the underlying insult may lead to significant improvements in disease sequelae.
