Localized IgG4-related disease manifested on the tongue: a case report

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs. IgG4-RD may show a variety of initial symptoms. In the oral mucosa, lesions present as inflammatory fibrosis with a large number of IgG4-positive plasma cells. Evaluating treatment is a well-known problem in IgG4-RD due to the absence of an established assessment system. There are difficulties in defining the severity of the disease, which is why treatment is primarily based on its clinical manifestations.
 We present a case report of localized IgG4-RD with ulcerative and proliferative manifestations on the tongue, which clinically mimicked oral squamous cell carcinoma. A tumor-like lesion on the tongue can indicate something else other than the malignant or reactive changes commonly found in the oral mucosa. Multiple differential diagnoses of these atypical oral lesions, including localized IgG4-RD, should be considered.

Oral and maxillofacial manifestations of IgG4‐related disease: A clinicopathological study

“…Currently, an immunohistochemical IgG4+ to IgG+ cell ratio higher than 40% has a better diagnostic value than absolute numbers of IgG4+ cells per 40× high‐power fields 21 . Eventually, IgG4‐RD may have an IgG4/IgG ratio < 40% 9 . Therefore, clinicians should consider a combination of clinical, serologic, radiologic, and pathologic/immunohistochemical features when trying to ascertain the diagnosis of IgG4‐RD.…”

“…21 Eventually, IgG4-RD may have an IgG4/IgG ratio < 40%. 9 Therefore, clinicians should consider a combination of clinical, serologic, radiologic, and pathologic/immunohistochemical features when trying to ascertain the diagnosis of IgG4-RD. For instance, our case #6 had an IgG4/IgG ratio lower than 40%, but still met the criteria for the diagnosis of IgG4-RD.…”

“…The clinical manifestations of this disease are extremely variable. Virtually any organ can be involved, but the most commonly involved organs are the pancreas, kidneys, orbital adnexal structures, salivary glands, and retroperitoneum 4,8,9 . As it is a multiorgan disease, four distinctive phenotypes were described according to organ involvement: (I) pancreaticobiliary disease, (II) retroperitoneal fibrosis with or without aortitis, (III) head and neck‐limited disease, and (IV) classic Mikulicz's syndrome with systemic involvement 10 …”

“…Virtually any organ can be involved, but the most commonly involved organs are the pancreas, kidneys, orbital adnexal structures, salivary glands, and retroperitoneum. 4,8,9 As it is a multiorgan disease, four distinctive phenotypes were described according to organ involvement: (I) pancreaticobiliary disease, (II) retroperitoneal fibrosis with or without aortitis, (III) head and neck-limited disease, and (IV) classic Mikulicz's syndrome with systemic involvement. 10 The head and neck region is the second most common site for IgG4-RD manifestation 11 and a variety of anatomical structures can be affected, including the orbits, meninges, ears, thyroid, skull bones, sinuses, and major salivary glands, 12 whereas the involvement of the oral cavity is extremely rare.…”

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

“…Lesions at this site exhibited a variegated pattern, with nodular masses resembling plasma cell mucositis or oral cancer, 32 submucosal nodules similar to IPT, 33 or ulcerations progressively evolving into polypoid neoformations. 34 All the subsites of the tongue were involved, but most notably the lateral borders and the ventral aspects.…”