PPP syndrome

Last revised by Yuranga Weerakkody on 3 Jun 2016

PPP syndrome is the extremely rare association of pancreatitis, panniculitis, and polyarthritis.

Most commonly affects middle-aged male patients with a history of heavy alcohol use 1,2

In the majority of cases, abdominal symptoms are mild or absent, making correct diagnosis challenging 1,2

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

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