Plasma cell granuloma of the oral cavity—a mucosal manifestation of immunoglobulin G4-related disease or a mimic?

Abstract

The aim of the study was to test the hypothesis that oral plasma cell granuloma may represent a mucosal manifestation of immunoglobulin (Ig)G4-related disease (IgG4-RD) in the oral cavity. The study sample comprised two males and four females, aged 54-79 years (median 62 years). The lesions were localized on gingival/alveolar mucosa (four cases), hard palate, and floor of the mouth, measuring 17-40 mm (median 31 mm). The duration of the lesions ranged from 3 months to several years. Information on IgG4 serum levels was available for two patients, and these were increased to 1.85 and 1.65 g/L, respectively. The follow-up period ranged 11-30 months (median 13 months). None of the lesions recurred, and none of the patients developed any manifestation of IgG4-RD. Microscopically, all cases presented as nodular lesions composed of numerous polyclonal plasma cells admixed with lymphocytes, histiocytes, mast cells, and eosinophils, set within collagenized stroma in variable proportions. Obliterative phlebitis was observed in two cases. The number of IgG4-positive plasma cells ranged between 51 and 142 per HPF (median 114), while the IgG4/IgG ratio values ranged between 0.16 and 0.72 (median 0.44) and were above 0.40 in three cases. Based on international criteria, two cases were diagnosed as definite and one as probable IgG4-RD. Oral plasma cell granuloma is a heterogeneous group of lesions, and a subset may represent a mucosal manifestation of IgG4-RD in the oral cavity.

IgG4-related disease in the head and neck region: report of two cases and review of the literature

“…In general, the IgG4 serum level is considered an unreliable diagnostic criterion. This is due to the fact that almost 40% of patients with a histologically confirmed IgG4-RD serum level of IgG4 fall within the normal range, particularly in cases where the disease is localized in one organ, as happened in the second case [9,10]. Conversely, in patients meeting criteria (a) and (b), a possible diagnosis of IgG4-RD can be made [7,8].…”

“…Upper respiratory tract presentation of the disease is rare (Table I) [3,8,9,11,12,13,14,15,16]. A review of the available literature showed only single cases of IgG4-RD of the oral cavity with presentation in the palate, tongue, floor of the mouth, and upper and lower gingiva [3,8,9].…”

“…The patient presented in Case 2 was younger, although Melo et al presented a case of IgG4-RD of the submandibular gland in an 11-year-old boy [21]. In patients with confirmed IgG4-RD, involvement of other organs should be excluded [9,10]. Patients with systemic IgG4-RD require a multidisciplinary approach that includes rheumatologists, gastroenterologists, pathologists, radiologists, and surgeons [22].…”

IgG4‐positive plasma cells are more often detected in chronic periapical lesions arising from permanent rather than primary teeth

“…In the current study, IgG4 expression, similar to that of IgA, was higher in chronic periapical lesions arising from permanent compared with primary teeth and suggests that these differences may be involved, at least in part, in the extracellular matrix remodelling mechanisms in chronic periapical lesions. In the oral cavity, several studies have described IgG4+ plasma cell infiltration in specific and nonspecific plasma cell-rich chronic inflammatory lesions (Strehl et al 2011, Andrew et al 2014, Cottom et al 2015, Laco et al 2015. Moreover, a single study has assessed chronic periapical lesions, without distinguishing between radicular cyst or periapical granuloma (Strehl et al 2011) and reported that the mean IgG4/IgG plasma cell ratio was 32%.…”

Cross-segment spinal plasma cell granuloma:a case report

“…One case study even reported amlodipine-induced PCG of the gingiva, revealing the potential contribution of drug-cellular interaction in the pathogenesis of this entity [18]. Recent studies supported PCG as a part of IgG4-related disease [3,[19][20][21][22][23], which was defined as a combined presence of the increased numbers of IgG4 positive plasma cells and the characteristic histopathological appearance. The histopathological features included a dense lymphoplasmacytic infiltration, a storiform pattern of fibrosis, and an obliterative phlebitis [24].…”