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Published on: December 15, 2011
Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report
Nasser M AlMadan1, Mohammed K Alwhabi2, Assem S Assem3
1Oral Medicine, Prince Sultan Military Medical City, Riyadh, SAU.
Abstract:
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.
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