Secondary Logo

Journal Logo

Institutional members access full text with Ovid®

IgG4-Related Skin Disease Presenting as a Pseudolymphoma in a White Adolescent Girl

Shakeri, Aria HBSc*; Kindley, Kimball Jade MD; Noland, Mary Margaret MD; Gru, Alejandro Ariel MD

The American Journal of Dermatopathology: September 2019 - Volume 41 - Issue 9 - p 675–679
doi: 10.1097/DAD.0000000000001401
Extraordinary Case Report

Abstract: Immunoglobulin gamma (Ig) type 4–related disease (IgG4-RD) is a chronic immunologic systemic disorder that has gained worldwide recognition in the past decade. This entity can affect almost every organ system, and its characteristic lesions have been found in a variety of organs such as lacrimal and salivary glands, pancreas, liver, bile ducts, lungs, kidneys, retroperitoneum, breast, aorta, thyroid, and prostate. This case reports a very rare case of IgG4-RD presenting with a cutaneous lesion in a young female patient mimicking a lymphoproliferative disorder. IgG4-RD affecting the skin is a rare entity and has mostly been reported in Japanese men of middle to older age. IgG4-RD with cutaneous involvement should be in the differential of non-neoplastic, lymphomas, autoimmune, and infectious disorders of the skin.

*University of Virginia School of Medicine, Charlottesville, VA;

Department of Dermatology, University of Virginia School of Medicine, Charlottesville, VA; and

Department of Pathology, University of Virginia School of Medicine, Charlottesville, VA.

Correspondence: Aria Shakeri, HBSc, University of Virginia School of Medicine, 1215 Lee Street, 22908, Charlottesville, VA (e-mail:

A. A. Gru has a consultant and advisory board relationship with Seattle Genetics and BMS. The remaining authors declare no conflicts of interest.

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.