Korean J Gastroenterol.  2019 Sep;74(3):175-182. 10.4166/kjg.2019.74.3.175.

Pancreatitis, Panniculitis, and Polyarthritis Syndrome Simulating Cellulitis and Gouty Arthritis

• Kim EJ
• Park MS
• Son HG
• Oh WS
• Moon KW
• Park JM
• Kang CD
• Lee S

• KMID: 2458699
• DOI: http://doi.org/10.4166/kjg.2019.74.3.175

Abstract

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.

Keyword

Pancreatitis; Panniculitis; Arthritis; Pancreatic neoplasms

MeSH Terms

Abdominal Pain
Anti-Inflammatory Agents
Arthritis*
Arthritis, Gouty*
Biopsy
Carcinoma, Acinar Cell
Cellulitis*
Diagnosis
Diagnostic Errors
Fever
Foot
Humans
Middle Aged
Mortality
Pancreatic Neoplasms
Pancreatitis*
Panniculitis*
Skin
Tomography, X-Ray Computed
Anti-Inflammatory Agents

Figure

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  Fig. 1 Gross appearance of the left foot showing erythematous swelling and multiple erythematous nodules in both pretibial regions.

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  Fig. 2 Computed tomography of the abdomen demonstrating decreased volume of the pancreas, dilatation of the main pancreatic duct (white arrowhead), and low attenuated lesions with surrounding irregular infiltration in the left anterior pararenal space, suggesting chronic pancreatitis and superimposed peripancreatic necrosis, respectively.

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  Fig. 3 Erythematous swelling of the second proximal interphalangeal and fifth metacarpophalangeal joint of the right hand.

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  Fig. 4 Plain radiographs of the right hand. (A) On hospital day 6, soft tissue swelling without a bone abnormality in the second proximal interphalangeal and fifth metacarpophalangeal joints (hollow arrowheads) is evident. (B) On hospitalization day 58, moth-eaten bone destruction is visible at the bases of the second middle and fifth proximal phalanges (white arrowheads).

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  Fig. 5 Histopathology examination of the lesion showed mostly lobular panniculitis without vasculitis, with adipocyte necrosis (“ghost cells,” black arrowheads) surrounded by neutrophils (H&E, ×100).

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  Fig. 6 Technetium-99m hydroxymethylene diphosphonate bone scan showing symmetrical uptake in the foot, hand, wrist, and elbow joints.

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