Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report

Rosai-Dorfman disease (RDD) is a rare condition characterized by the proliferation of non-Langerhans cell histiocytes that are associated with phagocytosed lymphocytes (emperipolesis). Clinically, it is classified into nodal, extra-nodal, neoplasia-associated RDD, and immune-related. Here, we present a case of a 65-year-old female who presented with facial pain following a dental procedure with no focal neurologic deficit. The MRI of the head and neck showed a well-defined lobulated soft tissue lesion with homogenous enhancement over the left cheek. Excision of the lesion was done, and the histopathological study reported extra-nodal RDD with features of IgG4-related sclerosing disease. The patient had no recurrence over the two years from the date of diagnosis.