Context.—

Immunoglobulin G4 (IgG4)–related disease is rare but well characterized in adults; however, the clinical and histologic manifestations in children may differ.

Objective.—

To review the clinical and histologic features of IgG4-rich head and neck lesions in a pediatric population.

Design.—

Retrospective search for cases with IgG4 immunohistochemical staining performed at our institution from 2011 to 2019. Review of clinical courses, serology profiles, histologic patterns, and immunohistochemical staining patterns.

Results.—

Four pediatric IgG4-rich lesions were identified and showed distinct histologic patterns from adult IgG4-related disease, including absence of pathognomonic findings associated with the latter. One case showed intralesional immunoglobulin light-chain restriction. Clinical review showed serum IgG4 elevation in 2 of 4 cases, presence of additional autoantibody positivity, and a generally benign/treatment-responsive clinical course.

Conclusions.—

Pediatric IgG4-related disease shows distinct clinical, serologic, and histologic features from its adult counterpart. Pediatric IgG4-related disease involving the orbit has unique clinical characteristics, including frequently normal serum IgG4 levels and female predominance. Awareness and evaluation for these features may improve diagnosis and treatment.

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Author notes

A portion of this work was supported by a Nationwide Children's Hospital Department of Pathology and Laboratory Medicine Pilot/Feasibility Research Grant.

The authors have no relevant financial interest in the products or companies described in this article.

Part of this work was presented as a platform presentation at the Society for Pediatric Pathology/Paediatric Pathology Society Joint Meeting; October 19, 2019; Pittsburgh, Pennsylvania.