Rosai-Dorfman disease in an Asian Indian woman with classic generalized lymphadenopathy and nasal obstruction: a case report.
Rosai-Dorfman disease is rare. When it does occur, it usually affects children, and it has a propensity for whites and blacks as opposed to members of other races. We report the case of a 45-year-old Asian Indian woman who presented with painful masses in the area of the axillary, cervical, and inguinal lymph nodes. She had a decade-long history of tonic-clonic seizures and a recent history of digestive complaints and progressive nasal stuffiness. Endoscopic examination of the left nasal cavity revealed the presence of submucosal bulges along the septum and the lateral wall; hypertrophy of the adenoids was also noted. Histologic analysis of lymph node specimens revealed dilated parenchymal sinuses, germinal activity with infiltration of numerous histiocytes (emperipolesis), and chronic inflammatory cells. The patient was diagnosed with Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) with atypical extranodal involvement (the nasal area). An aggressive course of polychemotherapy thrice daily was initiated, but it had no lasting effect, and the patient died 8 months later of an undetermined cause. Our patient's age at the onset of her disease, her race, and the extranodal involvement make this case of Rosai-Dorfman disease unusual and perhaps unique.
Singh
Neetu
N
Division of Toxicology, Central Drug Research Institute, M.G. Marg, Lucknow-226001, UP, India. neetuaashi@yahoo.com
Sharma
Ramesh
R
Verma
Umesh P
UP
Singh
Sanjay K
SK
Dixit
Jaya
J
Balapure
Anil K
AK
D000234:Adenoids / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D015618:Histiocytosis, Sinus / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D006984:Hypertrophy / Q000473:pathology; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology; D008206:Lymphatic Diseases / Q000150:complications* / Q000175:diagnosis*; D008875:Middle Aged; D015508:Nasal Obstruction / Q000150:complications* / Q000175:diagnosis*; D009418:S100 Proteins / Q000235:genetics / Q000378:metabolism
D009418:S100 Proteins