The "great imitator": IgG4-related disease of the oral cavity. Two case reports and scoping review

Head Neck. 2024 Jun;46(6):1510-1525. doi: 10.1002/hed.27763. Epub 2024 Apr 3.

Abstract

This study aimed to review the lesser-known intraoral manifestations of immunoglobulin G4-related disease (IgG4-RD). In this paper we report an unprecedented case of oral IgG4-RD mimicking angiolymphoid hyperplasia with eosinophilia (ALHE), and another case presenting as plasma cell gingivitis. We then performed a scoping review of published cases of IgG4-RD involving the oral cavity. The following data were collected for each case: age, sex, intraoral site(s) involved, clinical appearance, imaging features, serum IgG4 values, histopathology, treatment, and follow-up duration. Fifty-one cases of oral IgG4-RD were published in literature. The hard palate and jaw bones were the two main locations reported, while the histological identification of a IgG4/IgG plasma cells ratio ≥40% was fundamental for diagnosis. Conversely, the pathological features of storiform fibrosis and obliterative phlebitis were not common. Future reports regarding oral IgG4-RD should report clear adherence to the recognized international diagnostic criteria of the disease.

Keywords: IgG4‐RD; IgG4‐related disease; angiolymphoid hyperplasia with eosinophilia; oral IgG4‐RD; plasma cell gingivitis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Angiolymphoid Hyperplasia with Eosinophilia / diagnosis
  • Angiolymphoid Hyperplasia with Eosinophilia / pathology
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G4-Related Disease* / diagnosis
  • Male
  • Middle Aged
  • Mouth Diseases / diagnosis
  • Mouth Diseases / pathology

Substances

  • Immunoglobulin G