PPP syndrome is the extremely rare association of pancreatitis, panniculitis, and polyarthritis.
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Epidemiology
Most commonly affects middle-aged male patients with a history of heavy alcohol use 1,2.
Clinical presentation
In the majority of cases, abdominal symptoms are mild or absent, making correct diagnosis challenging 1,2.
Pathology
PPP syndrome is caused by pancreatic disease (acute/chronic pancreatitis, pancreatic tumors, pancreatic ischemia) with systemic spillage of pancreatic enzymes and manifests as 1-3:
- lower limb skin lesions (may also be trunk or upper limbs)
- polyarthritis, most commonly affecting the metacarpophalangeal, knee, and ankle joints
- intramedullary fat necrosis