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)

Rosai-Dorfman disease (RDD) is a systemic disease and its etiology is not well understood. It is a very rare but established disease. We report a case of RDD diagnosed in the upper airway that became a life-threatening condition because of the stricture of the subglottic space. A 49-year-old female complained of nasal obstruction, hoarseness and lacrimation. After evaluation following by fiberscopic examination and CT scanning of the head and neck, sinusitis and laryngeal masses were detected. Endoscopic sinus surgery and endolaryngeal microsurgery were performed simultaneously. The masses in the nasal cavity and larynx showed the same histological findings. Proliferative large histiocytes with voluminous clear cytoplasm and rounded nuclei, and lymphophagocytosis known as emperipolesis are the characteristic features of RDD. In this case, extranodal manifestations in the subglottic space gradually emerged postoperatively. Tracheotomy was performed to ensure an airway and prevent suffocation. An extensive treatment is not required in most patients with RDD, however, some patients who have extranodal manifestations involving the airway may detoriorate into a life-threatening condition.
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PMID:Rosai-Dorfman disease revealed in the upper airway: a case report and review of the literature. 1536 64

Sinus histiocytosis with massive lymphadenopathy is also known as Rosai-Dorfman disease (RDD) and is characterized by painless bilateral cervical lymphadenopathy. In the present case report, a 67-year-old Chinese woman presented with a 3-month history of progressive voice hoarseness, progressive dyspnea on exertion and a foreign body sensation. MRI revealed a lesion involving the right side of the paraglottic space. The lesion was totally resected. Based on the histologic features and immunoreactivity for the S-100 protein and CD68, a diagnosis of RDD was made. We described an extremely unique case of RDD that was observed in the paraglottic space and discussed its clinical and histopathologic features, differential diagnoses and treatment options.
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PMID:Rosai-Dorfman disease (RDD) in the paraglottic space: report of a case and review of literature. 2672 69

We report a 38-year-old man who presented with bilateral conjunctival congestion, hoarseness, and progressively growing pruritic, infiltrated skin lesions that had first begun over the face and neck, and later spread to the trunk and the limbs in 4 months. The clinical appearance of the lesions mimics granulomatous rosacea, acne vulgaris, or pityrosporum folliculitis. Histopathologic examination of the lesions from the face and chest both revealed dense dermal nodular lymphohistiocytic infiltrates which were positive for CD68 and S-100, but negative for CD1a. A systemic work-up for him detected no lymphadenopathy or other systemic involvement. A diagnosis of extranodal Rosai-Dorfman disease was made, and the patient received systemic glucocorticoids, with considerable improvement after 4 months of therapy.
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PMID:Granulomatous rosacea-like skin rash: extranodal Rosai-Dorfman disease. 3226 95