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 patient with steroid responsive cutaneous Rosai-Dorfman disease (RDD) associated with bilateral anterior uveitis and hypothyroidism. RDD is a rare histiocytic disorder of unknown aetiology. There is characteristic histology in which large pale-staining histiocytes exhibit emperipolesis and stain positive for S-100 protein. This condition is thought to be a reactive process rather than a neoplastic one and viral and immune mediated causes have been postulated.
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PMID:Steroid responsive cutaneous Rosai-Dorfman disease associated with uveitis and hypothyroidism. 1213 69

A 73-year-old woman presented with mild anterior uveitis, ipsilateral optic neuropathy, and ipsilateral skin nodules. A compressive mass at the level of the orbital apex and sphenoid wing was found on cranial magnetic resonance imaging. Biopsy of the skin nodules revealed histopathologic evidence of sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease (RDD). Systemic investigations failed to show any massive lymphadenopathy, making this a case of extranodal RDD. This is a salient case in that it proposes three simultaneous and separate sites of involvement by extranodal RDD. It also exemplifies that RDD should be a suspect diagnosis even in the absence of lymphadenopathy.
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PMID:Rosai-Dorfman disease. Extranodal sinus histiocytosis in three co-existing sites. A case report. 1262 52

A 15 year-old-male presented with concurrent sinonasal polyposis and anterior uveitis with cervical lymph node enlargement. Cervical lymph node biopsy confirmed Rosai-Dorfman disease. Though patient's eye symptoms did not respond well to topical corticosteroid treatment but he showed a remarkable regression in the size of nasal polyps and cervical lymph nodes after systemic corticosteroids. Intraocular involvement in such a case is rare.
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PMID:Rosai Dorfman syndrome with sinonasal mucosa and intraocular involvement. 1787 2