Locally destructive mandibular pseudotumor as a manifestation of immunoglobulin G4–related disease

The “great imitator”: IgG4‐related disease of the oral cavity. Two case reports and scoping review

“…A moderate-to-dense inflammatory infiltrate mainly constituted of lymphocytes and plasma cells represents a constant feature, but it should be reminded that in IPT-like masses, where the fibrosclerosing process may be advanced, the infiltrate is less pronounced and may be detected only in surrounding tissues. 27 Two important features regarded as key elements in histopathology of IgG4-RD are storiform fibrosis and obliterative phlebitis. 2 Storiform fibrosis is a swirling, "cart-wheel" pattern of fibrosis which may have a patchy distribution and is commonly observed in IgG4-RD, including those cases affecting the submandibular gland.…”

“…69 IgG4-RD is a condition that requires medical treatment, with the first-line therapy represented by systemic corticosteroids (i.e., oral prednisone), but in those patients with long-standing sclerosing lesions or IPT-like masses, surgery may be necessary before starting corticosteroid treatment to recover. 27 Rituximab, an anti-CD20 antibody employed also in other autoimmune disorders, is an effective second-line therapy adopted in nonresponsive cases, in patients with multiorgan involvement or contraindications to prolonged steroid therapy. 70 In conclusion, this paper presents for the first time an oral case of IgG4-RD mimicking ALHE.…”

“…39 CT scans of lesions involving the jaw bones only reinforced the suspicion of malignancy or infection, rather than identifying features that could make clinicians lean to a diagnosis of IgG4-RD. 27 MRI helped to better visualize the pseudotumors in soft tissues, but a nonhomogeneous signal on T2-weighted slides and an irregular enhancement after cmi were commonly reported, corroborating thus the suspicion of a neoplastic disease. 35 The only diagnostically relevant imaging technology, as for other body sites, was 18F-FDG PET/CT total body scan.…”

“…27,28 Interestingly, several reports described the presence of a nonhealing postextraction alveolar socket with evidence of exuberant granulation tissue. 29,30 Conversely, only two cases were described in the mandible, one resembling IPT, 27 and the other one similar to a large ossifying fibrous epulis accompanied by marginal resorption of the alveolar bone. 31 The tongue was the third most affected location in the oral cavity, tied for incidence with buccal mucosa (6/51, 11.8%).…”

“…In this region, IgG4-RD mostly appeared as an inflammatory pseudotumor (IPT) accompanied by bone destruction, and sometimes extending to the adjacent structures (i.e., hard palate, pterygopalatine fossa, cheek and/or masticatory muscles). 27,28 Interestingly, several reports described the presence of a nonhealing postextraction alveolar socket with evidence of exuberant granulation tissue. 29,30 Conversely, only two cases were described in the mandible, one resembling IPT, 27 and the other one similar to a large ossifying fibrous epulis accompanied by marginal resorption of the alveolar bone.…”