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)

A 49-year-old Japanese woman was found to have innumerable, small sessile elevations throughout the stomach as revealed by radiographic and endoscopic examination. Endoscopic biopsy showed that the elevations were formed by a diffuse proliferation of histiocytes in the lamina propria mucosae. The histiocytes were characterized by abundant interdigitating cytoplasmic projections, Birbeck granules, an oval or indented nucleus, and an absence of phagocytosis. They stained for S-100 protein but not for lysozyme, alpha-1-antitrypsin, or nonspecific antigen cross-reacting with carcinoembryonic antigen. Five and a half years after the first diagnosis, most of the elevated lesions disappeared, but a few histiocytoid cells were noted microscopically. Systemic examination showed no abnormalities outside the stomach. The patient has continued to be well. This case was diagnosed as primary benign histiocytosis X of the stomach. Four similar cases have been reported. This is the first case that was diagnosed using biopsy techniques and in which the natural history--i.e., from multiple polypoid extension throughout the stomach to spontaneous remission--was observed.
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PMID:Primary benign histiocytosis X of the stomach. A report of a case showing spontaneous remission after 5 1/2 years. 198 67

Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. Our results suggested that xanthomatous lesions of the skin to be composed of the histiocytic proliferation of two different cell lineages, i.e. S100+lyso-NCA- T-zone histiocytes and S100-lyso+NCA+ tissue macrophages. Only lesions of histiocytosis X were composed of the former cells. It is suggested that these markers will be useful in determining the delineation of the histiocytic system on the basis of functional heterogeneity.
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PMID:Immunohistochemical study on cutaneous histioproliferative lesions. 282 48

Eosinophilic (Langerhans' cell) granuloma is most common in children and is unusual in the elderly. A cutaneous lesion as the sole manifestation of adult Langerhans' cell histiocytosis is rare. We report an 81-year-old man who presented with a noduloulcerative lesion on the lower lip. A punch biopsy suggested a lymphoepithelioma-like carcinoma. In the absence of immunohistochemistry, the large pale cells with atypical lobular nuclei and patent nucleoli, with an overlying ulceration, were interpreted as carcinoma cells. Definitive diagnosis was established after complete resection of the lesion aided with the use of immunohistochemistry. The proliferating cells were S100, CD1a and CD207 (langerin) positive and cytokeratin, epithelial membrane antigen, CD15, CD30, melan A and carcinoembryonic antigen negative. To our knowledge, eosinophilic (Langerhans' cell) granuloma of the lip has not been described. The case constituted a diagnostic challenge because of its unusual clinical presentation and its histopathological similarity to certain other benign and malignant entities. Because there was no other organ involved, the patient was treated with complete surgical excision without recurrence or other organ involvement in the 4-month follow-up period. Labial eosinophilic (Langerhans' cell) granuloma should be considered in the histological differential diagnosis in cases with atypical polymorphous infiltrates including large pale cells.
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PMID:Localized eosinophilic (Langerhans' cell) granuloma of the lower lip. A lesion that may cause diagnostic error. 1918 5