Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe a case of a 42-year-old woman with systemic xanthogranulomatosis who primarily manifested bilateral intraorbital tumors, generally effecting many regions. These included the intracranial dura mater, the spinal cord, the retroperitoneum, the pericardium, and the mediastinum. This disorder is basically similar to systemic Weber-Christian disease except for the absence of subcutaneous panniculitis. Immunohistochemical study reveals that this clinical entity is different from histiocytosis X because the origin of the foamy cells is not the T-zone histiocyte but the macrophage. The treatment of this disease is also discussed.
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PMID:[A case of systemic xanthogranulomatosis with an intracranial lesion: immunohistochemical study]. 202 77

The authors describe a 42-year-old woman with systemic xanthogranulomatosis and bilateral intraorbital tumors, who subsequently developed multiple lesions of the intracranial dura mater, spinal cord, retroperitoneum, pericardium, and mediastinum. Systemic xanthogranulomatosis is histologically similar to systemic Weber-Christian disease, except for the absence of subcutaneous panniculitis. Immunohistochemical studies suggest that this clinical entity can be differentiated from histiocytosis X, because foamy cells in systemic xanthogranulomatosis demonstrate macrophages but not T-zone histiocyte markers. Differentiation of this disease from other intracranial xanthogranulomas and treatment are discussed.
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PMID:An intracranial mass lesion in systemic xanthogranulomatosis: case report. 217 1