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The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells, sometimes called dendritic cell histiocytosis. These cells in combination with lymphocytes , eosinophils , and normal histiocytes form typical LCH lesions that can be found in almost any organ . [ 5 ]
Chronic multifocal Langerhans cell histiocytosis; Other names: Hand–Schüller–Christian disease: A child with Hand-Schüller-Christian Disease: Specialty: Dermatology: Symptoms: Triad of bulging eyes, breakdown of bone, diabetes insipidus [1] Other symptoms eg. bone pain, facial asymmetry, ear infections, teeth/gum problems, liver and lung ...
Mast cell: Mastocytosis: CD138: Plasma cell: Multiple myeloma Plasmablastic lymphoma: CD207 (Langerin) Langerhans cell: Langerhans cell histiocytosis: Chloracetate esterase (Leder) Mast cell: Mastocytosis: Chromogranin A: Merkel cell carcinoma: Cystokeratin 7: Paget's disease (breast and extramammary) Cytokeratin 20: Extramammary Paget's ...
Langerhans cell is represented by a yellow oval; blue arrows correspond to is_a relations, and orange arrows correspond to develops_from relations. Only a subset of Langerhans cell parent types are included in the figure. [1] A Langerhans cell (LC) is a tissue-resident macrophage of the skin [2] once thought to be a resident dendritic cell. [3]
Letterer–Siwe disease, (LSD) or Abt-Letterer-Siwe disease, is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH) and is the most severe form, involving multiple organ systems such as the skin, bone marrow, spleen, liver, and lung. Oral cavity and gastrointestinal involvement may also be seen.
Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis). It was declared a histiocytic neoplasm by the World Health Organization in 2016. [1]
Hashimoto–Pritzker disease, [1] ... Congenital self-healing reticulohistiocytosis is a condition that is a self-limited form of Langerhans cell histiocytosis. [2]: ...
Langerhans cells utilize E-cadherin to localize in the epidermis via homotypic interaction with E-cadherin expressed by keratinocytes. Histiocytomas lack expression of CD4 and Thy-1, which are consistently expressed by histiocytes in cutaneous and systemic histiocytosis.