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Dermatologic Disorders in 118 Patients with Autoimmune (Immunoglobulin G4-Related) Pancreatitis: A Retrospective Cohort Analysis

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Abstract

Background

Autoimmune pancreatitis is the prototypical manifestation of immunoglobulin G4-related disease, a fibroinflammatory syndrome that can affect virtually any organ. Rarely, skin involvement has been reported in immunoglobulin G4-related disease. Isolated case reports have described other distinct associated dermatoses.

Objective

Our objective was to determine the frequency and nature of dermatologic manifestations of immunoglobulin G4-related disease in patients with autoimmune pancreatitis.

Methods

Retrospective analysis of dermatologic conditions of patients with autoimmune pancreatitis.

Results

Among the 118 identified patients, 31 (26.3 %) had a dermatologic diagnosis [mean (standard deviation; SD) follow-up 5.9 (4.6) years]. Two patients (1.7 %) had necrobiotic xanthogranuloma; three (2.5 %) had another xanthomatous or xanthogranulomatous process. One patient (0.8 %) had pemphigus erythematosus. No patient had immunoglobulin G4-related skin disease.

Conclusion

Skin involvement in immunoglobulin G4-related disease appears to be rare. A disproportionately high number of patients had xanthomatous or xanthogranulomatous processes, including necrobiotic xanthogranuloma. It remains unclear whether the association between immunoglobulin G4-related disease and necrobiotic xanthogranuloma or other xantho(granulo)matous processes represents shared pathophysiology, a mutual underlying driver, or coincidence, though the results of this study cast doubt on the latter. Although pemphigus was not reported in immunoglobulin G4-related disease previously, the prominent role of the immunoglobulin G4 subclass in each condition makes this association intriguing.

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Acknowledgments

No sources of funding were used to conduct this study or prepare this manuscript. Dr Kindle, Dr Hart, Dr Chari, Dr Pittelkow, Dr Smyrk, and Dr Lehman have no conflicts of interest that are directly relevant to the content of this study.

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Authors and Affiliations

  1. Department of Dermatology, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA

    Scott A. Kindle & Julia S. Lehman

  2. Division of Anatomic Pathology, Mayo Clinic, Rochester, MN, USA

    Thomas C. Smyrk & Julia S. Lehman

  3. Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA

    Suresh T. Chari

  4. Department of Dermatology, Mayo Clinic, Scottsdale, AZ, USA

    Mark R. Pittelkow

  5. Division of Gastroenterology, Ohio State University, Columbus, Ohio, USA

    Philip A. Hart

Authors
  1. Scott A. Kindle
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  5. Thomas C. Smyrk
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  6. Julia S. Lehman
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Correspondence to Julia S. Lehman.

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Kindle, S.A., Hart, P.A., Chari, S.T. et al. Dermatologic Disorders in 118 Patients with Autoimmune (Immunoglobulin G4-Related) Pancreatitis: A Retrospective Cohort Analysis. Am J Clin Dermatol 16, 125–130 (2015). https://doi.org/10.1007/s40257-014-0109-1

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  • DOI: https://doi.org/10.1007/s40257-014-0109-1

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