Dermatologic disorders in 118 patients with autoimmune (immunoglobulin G4-related) pancreatitis: a retrospective cohort analysis.

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.

• Authors: Scott A Kindle, Philip A Hart, Suresh T Chari, Mark R Pittelkow, Thomas C Smyrk, Julia S Lehman
• Journal: American journal of clinical dermatology (Am J Clin Dermatol) Vol. 16 Issue 2 Pg. 125-30 (Apr 2015) ISSN: 1179-1888 [Electronic] New Zealand
• PMID: 25575835 (Publication Type: Journal Article)

Chemical References

• Immunoglobulin G

Topics

• Autoimmune Diseases (immunology)
• Female
• Humans
• Immunoglobulin G (immunology)
• Male
• Pancreatitis (immunology)
• Retrospective Studies
• Skin Diseases (immunology)