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May Case of the Month
Abstract: Pancreatic disease can manifest with extra pancreatic symptoms, and while panniculitis is a rare occurrence, it can be found in up to 3% of patients. Even more rare, the combination of pancreatitis, panniculitis, and polyarthritis, known as PPP syndrome, has been reported in fewer than 70 cases. PPP syndrome can mimic conditions such as sarcoidosis and other autoimmune diseases, resulting in incorrect treatment, delayed diagnosis, and higher mortality rates. Fortunately, in this case, the incidental finding of an elevated lipase level (>3000 units/L) among other studies lead to early diagnosis and treatment.
A 43-year-old man presented to the emergency department with worsening extremity pain that led to the inability to complete his activities of daily living. His physical examination showed tender erythematous nodules, migratory pain, and swelling in multiple joints, along with night sweats. Biopsy of skin lesions revealed liquefactive necrosis of the subcutis, fat necrosis with ghost adipocytes, early calcification, and neutrophilic infiltrate, consistent with lobular panniculitis. Labs revealed elevated lipase level (>3000 units/L) suspicious of pancreatitis of an unknown origin. An abdominal CT scan, Endoscopic retrograde cholangiopancreatography with endoscopic ultrasound, and fine needle biopsy highly suggested pancreatic adenocarcinoma as the culprit. Limb imaging revealed destructive joint changes, a pathologic fracture of the olecranon, and scattered lytic bone lesions. The patient started FOLFIRINOX treatment and continues to tolerate chemotherapy well, with ongoing positive responses on surveillance scans. This case underscores the diverse and potentially deceptive dermatological manifestations of pancreatic diseases, emphasizing the importance of a comprehensive diagnostic approach.
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