IgG4-related submandibular gland disease

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Synonyms: Küttner tumor

A rare IgG4-related disease characterized by a benign tumor-like chronic inflammatory lesion of the submandibular gland. Histologic features are periductal fibrosis acinar atrophy obliterative phlebitis dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and formation of lymphoid follicles. Lobular architecture is preserved. Patients most commonly present with unilateral painless swelling of the submandibular gland. Serologic analysis reveals elevated IgG4 levels.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version July 2024

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