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

Rosai-Dorfman disease (RDD) with thyroid involvement is extremely rare; we herein present the sixth known case with a review of the previously reported five cases and discuss the clinicopathological characteristics of this entity. RDD with thyroid involvement has occurred only in females with a mean age of 56.3 years, which is significantly different from nodal RDD predominantly seen in young males. Besides this, autoimmune thyroiditis is frequently associated in RDD with thyroid involvement, which raises a suspicion of a common pathogenesis of both entities. RDD with thyroid involvement is commonly misdiagnosed preoperatively as thyroid malignancy with lymph node metastasis, which may lead to overtreatment and postoperative complication. Clinical presentations might be helpful for a preoperative differential diagnosis; while thyroid function, sonography, thyroid isotope scan, and fine needle aspiration have been less useful. All six cases were treated by surgery and complete remission was seen. However, two patients with a preoperative diagnosis of benign disease suffered from hypothyroidism due to total thyroidectomy.
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PMID:Thyroid involvement in Rosai-Dorfman disease. 1754 78

Soft tissue Rosai-Dorfman disease (STRDD) is rare, previously reported only as single cases and few series. Simian virus 40 (SV40), a polyomavirus, has been identified in lymphoid processes and has a controversial role in neoplasia etiology. Occasional cytoplasmic pink granular inclusions and nuclear changes led us to explore a viral etiology. Only unpublished STRDD from our files with adequate material, soft tissue location, and diagnostic confirmation were included. Immunohistochemistry and follow-up were obtained. Eighteen STRDD patients, 4 male and 14 female, had 29 lesions; 5 with 2 or more lesions. Ages ranged from 8 to 81 years (mean 42.6 years and median 42.5 years). Soft tissue Rosai-Dorfman disease locations include trunk or proximal extremity (n = 19), distal extremity (n = 5), "abdominal" (n = 3), face (n = 1), and unknown subcutaneous site (n = 1). Sizes ranged from 0.5 to 13.7 cm (median, 2.4 cm). Previous disease included lymphoma, buttocks injection site, diabetes and hypothyroidism, and radiation for chronic dermopathy. No patients had a preceding or concurrent known viral infection; none had lymphadenopathy at present. None were known to be immunocompromised. Soft tissue Rosai-Dorfman disease was rapidly progressing. Initial pathologic diagnosis ranged from Rosai-Dorfman disease or inflammatory pseudotumor to inflammatory malignant fibrous histiocytoma. Grossly STRDDs were multilobulated, tan-yellow, and firm; morphologically, circumscribed, and subcutaneous-based. All had sheets of polygonal histiocytes with abundant pale eosinophilic cytoplasm, emperipolesis, plasma cells, and lymphocytes scattered and within clusters. Focal spindle cell change and mild pleomorphism were each observed in 3 patients; 2 had focal necrosis, none with mitoses. Small granular pink cytoplasmic inclusions and nuclear viral-like changes were observed. By immunohistochemistry, all STRDDs were positive for S100 protein, negative for CD1a, Epstein-Barr virus, and latent membrane protein, yet 3 (all abdominal, 1 multicentric) of the 9 studied were focally positive for cytoplasmic and nuclear SV40 polyomavirus. All were treated by local excision. Follow-up on 14 patients older than 8 to 16 years revealed recurrence in 3 patients with persistent multiple lesions, one with abdominal location. There were no metastases or death from disease. Soft tissue Rosai-Dorfman disease is a rapidly evolving, mostly solitary and nonrecurrent trunk and proximal extremity subcutaneous lesion in middle-aged females. More than one third can have persistent multicentric disease. It is important to recognize STRDD, to separate it from malignancy. Epstein-Barr virus/latent membrane protein was negative but polyomavirus was positive in 3 patients with abdominal STRDD, one with multicentric persistent disease. The relationship of polyomavirus to the evolution of abdominal STRDD should be further explored.
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PMID:Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. 2085 Jun 91