Miscellaneous
Pancreatitis, Panniculitis, and Polyarthritis

https://doi.org/10.1016/j.semarthrit.2008.10.001Get rights and content

Background and Objective

Lobular panniculitis, together with polyarthritis and intraosseous fat necrosis, may occasionally complicate pancreatic disease. This triad is known in the literature as the pancreatitis, panniculitis, and polyarthritis (PPP syndrome). We describe a case of the PPP syndrome and review the available literature to summarize the clinical characteristics of patients with this condition.

Methods

A patient with the PPP syndrome, with evidence of extensive intraosseous fat necrosis in the joints involved revealed by magnetic resonance imaging, is described and the relevant literature based on a PubMed search from 1970 to February 2008 is reviewed. The keywords used were pancreatitis or pancreatic disease, panniculitis, arthritis, and intraosseous fat necrosis.

Results

Including our case, 25 well-documented patients with the PPP syndrome have been reported. Our patient had few abdominal symptoms despite high serum levels of pancreatic enzymes. In our review of the literature, almost 2/3 of patients had absent or mild abdominal symptoms, leading to misdiagnosis. The delay in diagnosis and specific treatment of the underlying pancreatitis worsens the prognosis of this condition, which has a mortality rate as high as 24%.

In nearly 45% of the patients, the arthritis follows a chronic course with a poor response to nonsteroidal anti-inflammatory drugs and corticosteroids, and the rapid development of radiographic joint damage.

Conclusion

Certain forms of pancreatic disease can very occasionally cause arthritis and panniculitis. Although uncommon, physicians should be alert to the possible presence of this syndrome for 2 reasons: first, unrecognized pancreatic disease can be fatal if not treated promptly; second, to avoid inappropriate and risky therapy to improve joint symptoms.

Section snippets

Methods

In addition to our case, a literature search (PubMed database, National Library of Medicine, Bethesda, MD) for articles published between January 1970 and February 2008 was performed using the Medline subheadings and key words “pancreatitis” or “pancreatic disease,” “panniculitis,” “arthritis,” and “intraosseous fat necrosis.” Only English-, French-, and Spanish-language reports were selected for review. The references of the studies obtained were then examined to identify additional reports.

Case Report

A 45-year-old man with a 30-year history of heavy alcohol abuse was admitted to our center for alcohol detoxification. On admission, the patient reported that he had experienced mild upper abdominal pain, without nausea or vomiting, for 1 week.

On physical examination the abdomen was soft, tender in the epigastrium and left hypochondrium, with nontender hepatomegaly; joint and skin examination at that time were normal. Laboratory examination revealed an elevated erythrocyte sedimentation rate

Discussion

Lobular panniculitis, together with arthritis and/or bone necrosis, may complicate pancreatic disease. This triad has been described mainly in patients with acute or chronic pancreatitis (2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24), as well as in a few cases of pancreatic tumors (25, 26) and ischemic pancreatic disease (27). The acronym PPP syndrome is not widely used and is not entirely accurate as the arthritis is not always polyarticular.

Although this

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