Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of extranodal Rosai-Dorfman Disease (RDD) of the scophoid in a 52-year old female. The patient presented with pain, swelling, and tenderness on deep palpation of the left wrist. Clinicoradiological diagnosis was osteomyelitis or tenosynovitis and curettage was performed on the lytic lesion over scaphoid to procure tissue. Diagnosis was made by histomorphology supported by immunostaining. The patient was managed conservatively with resolution of the lesion.
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PMID:Extranodal Rosai-Dorfman disease in a carpal bone. 2291 28

Rosai-Dorfman disease is a rare benign histiocytic disorder that arises predominantly in lymph nodes with fever and malaise, but can affect various organs, with or without lymphadenopathy. Solitary extranodal skeletal lesions are extremely rare. Herein, we describe a case of isolated disease of thoracic spine, with vertebral body fracture. A 14-year-old girl presented with cervical back pain for about one month as the only symptom. There was no lymphadenopathy. Results of laboratory tests were within reference ranges. The radiological examinations revealed collapse, osteolytic lesion and compressive fracture, which reduced vertical diameter of C4 vertebral body. The patient made a complete recovery after a limited C4 laminectomy, spondylodesis C3/C5 vertebra and biopsy of the lesion. Histologically, nonspecific cellular milieu and atypical histiocytic proliferation with emperipolesis, with CD68 and S100 positivity, confirmed a diagnosis of Rosai-Dorfman disease. Isolated extranodal skeletal Rosai-Dorfman disease should be considered among the differential diagnoses of similar changes, such as Langerhans cell histiocytosis, Erdheim-Cheister disease, eosinophilic granuloma, osteomyelitis, metastasis and lymphoma.
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PMID:Rosai-Dorfman disease of vertebra: Case report and literature review. 2862 Nov 4