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)

ICAM-3 is a newly recognized adhesion molecule, which is a member of the immunoglobulin supergene family of ICAMs, and has been shown to be identical with the CD50 antigen. Recent functional studies have shown that ICAM-3 is a ligand for LFA-1, and plays an important part in immune reactions. To date, very few data exist in the literature concerning its expression in the skin. In the present study, we investigated the expression of ICAM-3 in normal skin and in 98 biopsy specimens of various inflammatory and neoplastic dermatoses. ICAM-3 was found to be expressed by epidermal CD1a+ Langerhans cells, by cells of Langerhans cell histiocytosis, by T and B lymphocytes infiltrating the dermis in cutaneous lymphomas and in a wide spectrum of inflammatory dermatoses. Epidermal keratinocytes were consistently negative; endothelial expression of ICAM-3 was observed in six of the 98 cases. These results show that ICAM-3 is constitutively and widely expressed by cells participating in inflammatory dermatoses (including Langerhans cells and T and B lymphocytes), and that it can be, albeit rarely, induced on endothelial cells and dermal dendrocytes. These results highlight the important part that ICAM-3 may play in cutaneous inflammatory and immune reactions.
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PMID:Expression of ICAM-3/CD50 in normal and diseased skin. 854 91

Context.-We investigated expression of the adhesion molecule CD31 in sinus histiocytosis with massive lymphadenopathy (SHML) and Langerhans cell histiocytosis (LCH) because (1) SHML and LCH cells express a variety of cellular adhesion molecules and (2) SHML has been characterized as a reactive histiocytic proliferation, and tissue macrophages (histiocytes) are known to express CD31. Objective.-The purpose of this study was to determine whether SHML and LCH cells express CD31 and whether dual staining with CD31 and S100 facilitates diagnosis of these disease states. Methods.-Formalin-fixed, paraffin-embedded archival tissues were immunohistochemically stained via the labeled streptavidin-biotin method using antibodies against CD31 and S100 protein after heat-induced epitope retrieval. Archival tissues included SHML (n = 2), LCH (n = 10), malignant melanoma (n = 5), sinus hyperplasia (n = 4), granulomas (n = 4), granular cell tumor (n = 6), and normal skin (n = 4). Results.-Normal Langerhans cells in the epidermis were CD31(-)/S100(+); neoplastic Langerhans cells in LCH were CD31(+)/S100(+). Histiocytes in granulomas and in sinus hyperplasia were CD31(+)/S100(-); abnormal histiocytes in SHML were CD31(+)/S100(+). S100(+) tumors (malignant melanoma and granular cell tumor) were CD31(-). Conclusions.-The spectrum of cell types that express CD31 is expanded to include SHML and LCH. We speculate that up-regulation of CD31 in neoplastic Langerhans cells contributes to the migratory capability of LCH cells. CD31 may be a useful nonlysosomal marker of macrophages and their neoplastic counterparts (true histiocytic sarcomas). An immunohistochemical staining panel that includes CD31 and S100 facilitates the diagnosis of SHML and LCH.
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PMID:Sinus histiocytosis with massive lymphadenopathy and Langerhans cell histiocytosis express the cellular adhesion molecule CD31. 1265 80

Langerhans cell histiocytosis (LCH) is the unifying designation for a rare proliferative disorder that occurs predominantly in childhood and involves the main antigen-presenting cell of the epidermis. LCH can present in a multitude of ways, from a self-limited rash that resolves spontaneously to a systemic multi-organ disease with a 20% mortality rate. Because some forms behave in a relatively benign manner and are associated with an inflammatory cell infiltrate, it has been proposed that LCH might be a reactive disease. However, its neoplastic nature is suggested by the fact that the proliferating cells in LCH are clonal and overexpress p53. Nonetheless, no recurrent genomic, genetic or epigenetic abnormalities have been identified. Instead, a variety of molecular abnormalities that are consistent with disordered Langerhans cell maturation have been described. A faithful small animal model would aid our understanding of the pathophysiology of LCH but, to date, none exists. Challenges to the creation of a model include the lack of characteristically recurrent genetic abnormalities and the absence of a truly tissue-specific promoter to drive expression of genetic elements solely in Langerhans cells. Still, some of the phenotypic abnormalities in adhesion molecule or chemokine receptor expression might be modeled with sufficient precision to allow the testing of novel therapies.
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PMID:Langerhans cell histiocytosis: malignancy or inflammatory disorder doing a great job of imitating one? 1972 2