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)

Chronic lymphocytic leukemia (CLL) involving skin is a rare but well-documented occurrence, mainly reported in advanced disease. In contrast, CLL presenting with skin lesions is exceedingly rare, only few reports existing to date. We report a 70-year-old man who presented with two non-pruritic, papular lesions on the lower abdomen and proximal thigh. Biopsies showed dense lymphohistiocytic infiltrates involving the reticular dermis and subcutis without epidermotropism consisting mostly of small, CD20 and PAX-5-positive B-cells expressing CD5, CD23, CD43 and BCL2. Numerous large B-cells were present in a T-cell, histiocyte-rich background. A staging bone marrow biopsy showed a clonal B-cell proliferation with typical CLL flow cytometry immunophenotyping but neither lymphadenopathy nor absolute lymphocytosis was present. Numerous B and T-cell cutaneous lymphoproliferative disorders can be associated with increased numbers of histiocytes occasionally masquerading as benign disorders. This was the case with our patient's lesions, originally interpreted as cutaneous Rosai-Dorfman disease. A high index of suspicion from both the pathologist and the dermatologist is essential in identifying these rare but probably underrecognized occurrences of early systemic lymphoproliferative disorders presenting as cutaneous lesions with an unexpected cellular composition.
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PMID:Subclinical chronic lymphocytic leukemia with atypical cutaneous presentation. 1971 32

This report describes 2 new cases of testicular involvement by Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, and reviews the clinical and pathologic features of the other cases documented in the literature (4 cases) or included in the sinus histiocytosis with massive lymphadenopathy registry (7 cases). Our patients were 42 and 68 years of age, respectively, reflecting the usual middle-age of all the previously reported cases in this location, except for 1, which occurred in a child. Five cases were bilateral, and other sites, such as lymph nodes, kidney, skin, and adrenal gland, were involved in 8 cases. The histories were noteworthy in 2 of the cases, inasmuch as 1 patient had chronic lymphocytic leukemia, and another had a B-cell lymphoma. On gross examination, the testicular masses did not exhibit specific diagnostic features, whereas microscopic examination revealed the typical population of histiocytes, with abundant eosinophilic cytoplasm often exhibiting so-called emperipolesis. The differential diagnosis includes non-neoplastic conditions such as granulomatous orchitis, hematolymphoid disorders, a variety of metastatic tumors, and germ cell tumors.
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PMID:Involvement of the testis and related structures by Rosai-Dorfman disease: report of 2 new cases and review of the literature. 2406 26