IgG4-Related Disease Presenting as Inflammatory Midline Pseudotumor of the Face and Neck.

Authors

Rowena Lee Ying Kwan, Joyce Siong See Lee, Choon Guan Chua, Manish Mahadeorao Bundele, Jiun Yit Pan

Published in

International journal of dermatology. Oct 16, 2026. Epub Oct 16, 2026.

Abstract

A 78-year-old Chinese woman presented with a one-year history of a progressively enlarging purpuric rash over the central face and nasal dorsum, with subsequent development of a firm midline neck mass. Histopathology revealed dense lymphoplasmacytic infiltrate with fibrosis and obliterative phlebitis. Immunohistochemistry showed over 200 IgG4-positive plasma cells per high-power field with an IgG4-IgG ratio exceeding 40%, and serum IgG4 was markedly elevated at 3648.0 mg/L, confirming IgG4-related disease (IgG4-RD) with a head-and-neck limited phenotype. She responded well to prednisolone. This case illustrates a rare cutaneous presentation of IgG4-RD, which should be considered in differential diagnoses of head and neck lesions.

PMID:
42885337
Bibliographic data and abstract were imported from PubMed on 16 Oct 2026.

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