A young woman with steroid-responsive, IgG4-positive plasma cell-enriched cystic lymphangioma and chylous ascites

Alaa A Al Zaki; Shawna L Mann; Mollie N Carruthers; Graham W Slack; Luke Y C Chen

doi:10.1002/ccr3.1536

A young woman with steroid-responsive, IgG4-positive plasma cell-enriched cystic lymphangioma and chylous ascites

Clin Case Rep. 2018 Apr 17;6(6):1098-1100. doi: 10.1002/ccr3.1536. eCollection 2018 Jun.

Authors

Alaa A Al Zaki¹, Shawna L Mann², Mollie N Carruthers³, Graham W Slack⁴, Luke Y C Chen¹

Affiliations

¹ Division of Hematology Department of Medicine University of British Columbia Vancouver British Columbia Canada.
² Division of Nephrology Department of Medicine University of Western Ontario London Ontario Canada.
³ Division of Rheumatology Department of Medicine University of British Columbia Vancouver British Columbia Canada.
⁴ Department of Pathology and Laboratory Medicine University of British Columbia Vancouver British Columbia Canada.

Abstract

Lymphangiomas are benign tumors of the lymphatic vessels, which can be inflammatory and occasionally steroid-responsive. IgG4-related disease (IgG4-RD) is a recently defined fibro-inflammatory condition. We describe a novel association between reactive IgG4+ plasma cells and cystic lymphangioma in a young woman who had a dramatic clinical response to steroids.

Keywords: IgG4‐related disease; inflammation; lymphangioma; steroid.

Publication types

Case Reports