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)

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a self-limited histiocytic disorder of unclear etiology which most commonly presents with cervical lymphadenopathy. Purely extranodal presentation of RDD is uncommon, and isolated intralymphatic/intravascular confinement of this entity has not previously been described. We report a 16-yr-old female who presented with vaginal swelling and mass-like enlargement of the right labia. The mass had been present for nearly a year without pain or tenderness. Clinically, the lesion was thought to be a Bartholin gland cyst. Following surgical resection, histologic examination demonstrated a hypocellular myxedematous stroma with a mixture of ectatic thin and thick-walled vessels within which there were numerous collections of histiocytes, lymphocytes, and plasma cells. The histopathologic differential diagnosis included localized vulvar lymphedema, a specialized genital tract neoplasm, and childhood asymmetric labium majus enlargement. The histiocytes showed occasional plasma cells and lymphocytes within their cytoplasm, consistent with emperipolesis. Immunohistochemical studies showed that the histiocytes expressed CD163 and S100, while ERG and D2-40 highlighted their intralymphatic confinement, ultimately leading to the diagnosis of intralymphatic RDD. Intralymphatic RDD may present as vulvar lymphedema and can potentially mimic other myxedematous neoplasms of the vulvovaginal region.
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PMID:Intralymphatic Rosai-Dorfman Disease Associated With Vulvar Lymphedema: A Case Report of an Extremely Rare Phenomenon. 3127 98

Rosai-Dorfman disease (RDD) is an extremely rare benign histiocytic disorder that usually affects young adults. Extranodal involvement of the RDD is common and may occur in >40% of patients, but bone involvement occurs in <10% of cases. Furthermore, primary bone RDD is extremely rare. The present study reports a case of primary bone RDD arising in the infantile ilium. Plain radiographs and computed tomography (CT) revealed an osteolytic lesion at the peri-acetabular region of the patient's right ilium. Fluorodeoxyglucose positron emission tomography indicated an abnormal accumulation only in the right iliac bone, without any other accumulation. An open biopsy was performed and the diagnosis of primary RDD of bone in the ilium was made. The bone lesion exhibited spontaneous regression on radiography, and the patient was able to walk without any limping or pain at 8 months after the biopsy. After 18 months of follow-up, the bone lesion had completely disappeared, and no joint deformity was observed on radiography or CT. The present report described the clinicopathological details of this rare case and reviewed the relevant literature.
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PMID:Primary Rosai-Dorfman disease of bone arising in the infantile ilium: A case report. 3225 84

A 36-year-old female presented with pain and progressive decrease in the range of movements in the left elbow. There was contact with an open case of pulmonary tuberculosis. There were no constitutional symptoms except fever. X-ray showed periarticular osteopenia with destruction of the radial neck and head. MRI scan findings were also consistent with tuberculosis of the elbow. Histopathology examination confirmed the diagnosis of Rosai-Dorfman disease. The patient was treated with glucocorticoid and methotrexate and was asymptomatic after 18 months.
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PMID:Primary Rosai-Dorfman Disease of the Proximal Radius Masquerading as Tuberculosis of Elbow. 3248 8

A 47-year-old white man presented with a 14-month history of an asymptomatic 2-cm, slow-growing nodular lesion on his left shin that arose in the background of a black tattoo. An excisional biopsy followed by histological examination revealed a prominent lymphohistiocytic infiltrate, with many large, foamy histiocytic cells containing intact inflammatory cells within their cytoplasm, findings consistent with emperipolesis, a feature typical of Rosai-Dorfman disease (RDD). By immunohistochemistry, S-100 (a marker that is positive in almost all cases of RDD) was negative, arguing against the diagnosis of RDD. In addition, prominent black tattoo pigment was seen in many areas, expanding the differential diagnosis to include an unusual reactive lymphohistiocytic response to the tattoo mimicking RDD. Histologically, RDD shows many plasma cells, neutrophils, lymphocytes, and histiocytes with abundant foamy cytoplasm that contains intact lymphocytes and other cells, a phenomenon described as emperipolesis. A wide variety of cutaneous reactions to tattoos have been described, including tenderness, burning pain, inflammation, and pruritus. However, histologic features suggestive of RDD as a reaction to tattoo pigment have not been previously described and should therefore also be considered as a potential rare reaction pattern to tattoos.
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PMID:Rosai-Dorfman Disease-Like Reaction to Tattoo. 3256 40

Intracranial Rosai-Dorfman disease is quite rare. Here, we report a 67-year-old man with a 90 day history of pain and numbness in his right limbs. The patient was suspected of suffering from melanoma. Then he received craniotomy and was finally diagnosed with intracranial Rosai-Dorfman disease. MEDLINE was used to search the related literature; and the diagnosis, mechanism, treatment and prognosis of this rare tumor are discussed.
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PMID:Intracranial Rosai-Dorfman disease mimicking melanoma: a case report and review of the literature. 3266 85


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