Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a widespread papular eruption in a 5-year-old girl with rheumatic fever. Histological examination revealed a dense histiocytic infiltration in the dermis. On immunohistochemical studies, the cells were positive for vimentin, CD68, MAC387, alpha1-antichymotrypsin and lysozyme, but negative for CD1a and S-100 protein. Electron microscopic studies showed no Birbeck granules in their cytoplasm. A diagnosis of generalized eruptive histiocytoma of childhood was established. The skin lesions completely disappeared within 8 months.
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PMID:Generalized eruptive histiocytoma of childhood associated with rheumatic fever. 1052 34

Purely cutaneous Rosai-Dorfman disease is exceptional. The disease is characterized histologically by large, proliferating histiocytes exhibiting inflammatory cells within their cytoplasm (emperipolesis). We present here a case of purely cutaneous generalized disease in which the routine histopathology was suggestive of an inflammatory pseudotumor. Positivity for S-100 protein, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, Mac387 and CD68 proteins, and negativity for CD1a protein confirmed the diagnosis of Rosai-Dorfman disease. The rarity of this case lies in the presence of conspicuous inflammatory pseudotumor-like histopathologic changes, masking an otherwise typical sinus histiocytosis cell infiltrate. This unusual presentation of the disease requires a high index of suspicion by clinicians and pathologists.
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PMID:Cutaneous Rosai-Dorfman disease: histopathological presentation as inflammatory pseudotumor. A literature review. 1236 Nov 36