| Langerhans c.’s |
stellate dendritic cells, derived from precursors in the bone marrow, that appear clear on light microscopy and have a dark-staining, indented nucleus and characteristic inclusions (Birbeck granules) in the cytoplasm; they lack tonofilaments, desmosomes, and melanosomes. Langerhans cells are found principally in the stratum spinosum of the epidermis, but they also occur in other stratified epithelia and have been identified in the lung, lymph nodes, spleen, and thymus. They have surface markers characteristic of macrophages and are believed to be antigen-presenting cells involved in contact allergic responses and other cell-mediated immune reactions in the skin.
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| Langerhans cell g. |
see under histiocytosis.
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| Langerhans cell h. |
a generic term embracing a group of disorders characterized by proliferation of Langerhans cells (q.v.); children are more often affected than adults. Lesions may be unifocal or multifocal and may involve the bone marrow, endocrine system, or lungs (the last being more common in adults than in children). Although the cause is uncertain, these disorders are believed to arise from disturbances in regulation of the immune system. Called also eosinophilic granuloma or granulomatosis and Langerhans cell granulomatosis. Formerly called h. X.
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| Langerhans cell h., acute disseminated |
Letterer-Siwe disease.
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| Langerhans cell h., multifocal |
Langerhans cell histiocytosis occurring as erosive accumulations of proliferating Langerhans' cells, commonly within the medullary cavities of bones, but also affecting the skin, gingiva, lungs, and stomach. It most commonly affects children and is accompanied by seborrheic eruptions, fever, frequent occurrences of otitis media, mastoiditis, and upper respiratory tract infection, often with lymphadenopathy and splenomegaly. When the triad of calvarial bone defects, exophthalmos, and diabetes insipidus is present, it is referred to as Hand-Schüller-Christian disease.
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