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

Lorenzo Azzi; Francesca Magnoli; Diana Krepysheva; Federico Fontana; Andrea Coppola; Antonella Cappelli; Marta Dani; Paolo Battaglia; Dimitri Rabbiosi

doi:10.1002/hed.27763

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

Head Neck. 2024 Apr 3. doi: 10.1002/hed.27763. Online ahead of print.

Authors

Lorenzo Azzi^{1

2}, Francesca Magnoli³, Diana Krepysheva³, Federico Fontana^{1

4}, Andrea Coppola⁴, Antonella Cappelli⁵, Marta Dani^{2

6}, Paolo Battaglia^{6

7}, Dimitri Rabbiosi^{6

8}

Affiliations

¹ Department of Medicine and Technological Innovation, University of Insubria, Varese, Italy.
² Unit of Oral Medicine and Pathology, Dental Clinic, Azienda Socio-Sanitaria Territoriale dei Sette Laghi, Varese, Italy.
³ Unit of Pathology, Azienda Socio-Sanitaria Territoriale dei Sette Laghi, Varese, Italy.
⁴ Unit of Radiology, Azienda Socio-Sanitaria Territoriale dei Sette Laghi, Varese, Italy.
⁵ Unit of Rheumatology, Azienda Socio-Sanitaria Territoriale dei Sette Laghi, Varese, Italy.
⁶ Department of Biotechnology and Life Science, University of Insubria, Varese, Italy.
⁷ Unit of Otolaryngology and Head and Neck Surgery, Azienda Socio-Sanitaria Territoriale Lariana, San Fermo della Battaglia (CO), Italy.
⁸ Unit of Maxillofacial Surgery, Azienda Socio-Sanitaria Territoriale dei Sette Laghi, Varese, Italy.

PMID: 38566594
DOI: 10.1002/hed.27763

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

Review