Disordered Presentation of Paraneoplastic Pancreatitis, Polyarthritis and Panniculitis (PPP) Syndrome in a Patient with End-Stage Pancreatic Cancer
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Abstract
We report a rare case of PPP due to stage IV beta-islet pancreatic cancer presenting with polyarthritis, whose diagnosis was delayed due to an incomplete presentation of PPP syndrome. Following an extensive one-month infectious disease workup, tender erythematous papules began to develop, which prompted the consideration and subsequent biopsy for pancreatic panniculitis. Thus, the diagnosis was delayed due to a lagging cutaneous presentation—a component of the triad that is commonly the initial presentation in most patients.
References
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9. Price-Forbes AN, Filer A, Udeshi UL, Rai A. Progression of imaging in pancreatitis panniculitis polyarthritis (PPP) syndrome. Scand J Rheumatol. 2006;35(1):72-74.
2. Borowicz J, Morrison M, Hogan D, Miller R. Subcutaneous fat necrosis/panniculitis and polyarthritis associated with acinar cell carcinoma of the pancreas: a rare presentation of pancreatitis, panniculitis and polyarthritis syndrome. J Drugs Dermatol. 2010;9(9):1145-1150.
3. Zundler S, Erber R, Agaimy A, et al. Pancreatic panniculitis in a patient with pancreatic-type acinar cell carcinoma of the liver--case report and review of literature. BMC Cancer. 2016;16:130.
4. Fraisse T, Boutet O, Tron AM, Prieur E. Pancreatitis, panniculitis, polyarthritis syndrome: an unusual cause of destructive polyarthritis. Joint Bone Spine. 2010;77(6):617-618.
5. Garcia-Romero D, Vanaclocha F. Pancreatic panniculitis. Dermatol Clin. 2008;26(4):465-470, vi.
6. Requena L, Sanchez Yus E. Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol. 2001;45(3):325-361; quiz 362-324.
7. Narvaez J, Bianchi MM, Santo P, et al. Pancreatitis, panniculitis, and polyarthritis. Semin Arthritis Rheum. 2010;39(5):417-423.
8. Ferri V, Ielpo B, Duran H, et al. Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review. Int J Surg Case Rep. 2016;28:223-226.
9. Price-Forbes AN, Filer A, Udeshi UL, Rai A. Progression of imaging in pancreatitis panniculitis polyarthritis (PPP) syndrome. Scand J Rheumatol. 2006;35(1):72-74.