Pancreatic Panniculitis and Polyarthritis

  • Sebastian Zundler
  • Deike Strobel
  • Bernhard Manger
  • Markus F. Neurath
  • Dane Wildner
Orphan Diseases (B Manger, Section Editor)
  • 209 Downloads
Part of the following topical collections:
  1. Topical Collection on Orphan Diseases

Abstract

Purpose of Review

Polyarthritis can have numerous reasons and may thus constitute a challenge for differential diagnosis. One rare potential reason for sterile polyarthritis is underlying pancreatic disease with systemic hyperlipasemia, most often accompanied by painful skin lesions caused by a subcutaneous inflammatory process known as panniculitis. Systematic evidence on pancreatic panniculitis and polyarthritis is limited, particularly regarding its feature as facultative paraneoplasia with underlying intra- or even extra-pancreatic malignancy. Therefore, we will summarize the current knowledge about this orphan disease including epidemiological, pathophysiological, diagnostic, and treatment aspects in the present review.

Recent Findings

Although direct evidence is lacking, it is highly probable that pancreatic polyarthritis and panniculitis are caused by peripheral lipolytic activity of lipase systemically circulating due to benign (e.g., acute or chronic pancreatitis) or malign (e.g., acinar cell carcinoma (ACC) or adenocarcinoma) pancreatic disease. In the latter case, pancreatic polyarthritis and panniculitis are associated with poor outcome.

Summary

Pancreatic polyarthritis and panniculitis should always be included into diagnostic considerations, and once suspected, a thorough work-up to identify the underlying disease has to be performed.

Keywords

Pancreatic panniculitis Hyperlipasemia Polyarthritis Acinar cell carcinoma 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no competing interests.

Human and Animal Rights and Informed Consent

All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki Declaration and its amendments, institutional research committee standards, and institutional guidelines).

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Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  • Sebastian Zundler
    • 1
  • Deike Strobel
    • 1
  • Bernhard Manger
    • 2
  • Markus F. Neurath
    • 1
  • Dane Wildner
    • 1
  1. 1.Department of Medicine 1, University Hospital ErlangenFriedrich-Alexander-University Erlangen-NurembergErlangenGermany
  2. 2.Department of Medicine 3, University Hospital ErlangenFriedrich-Alexander-University Erlangen-NurembergErlangenGermany

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