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Juvenile xanthogranuloma is a form of histiocytosis, classified as non-Langerhans cell histiocytosis. [1] [2] It is a rare skin disorder that primarily affects children under one year of age but can also be found in older children and adults. [3] It was first described in 1905 by Adamson. [4]
Touton giant cells in a juvenile xanthogranuloma. H&E stain. Touton giant cells are a type of multinucleated giant cell observed in a myriad of pathological disorders and conditions. Specifically, Touton giant cells are found in lipid-rich lesions such as those of fat necrosis, xanthoma, xanthelasma and xanthogranulomas. Touton giant cells are ...
Non-Langerhans cell histiocytosis, also known as rare histiocytoses, comprise all histiocyte, macrophage, and dendritic cell proliferative disorders that are not categorized as hemophagocytic lymphohistiocytosis (HLH) or Langerhans cell histiocytosis (LCH).
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Xanthelasma in the form of XP can be diagnosed from clinical impression, although in some cases it may need to be distinguished (differential diagnosis) from other conditions, especially necrobiotic xanthogranuloma, syringoma, palpebral sarcoidosis, sebaceous hyperplasia, Erdheim–Chester disease, lipoid proteinosis (Urbach–Wiethe disease), and the syndrome of adult-onset asthma and ...
Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. [2] [6] [8] The disease can be either localized to the skin or involve other organs, as well. [2]
Non-X histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of monocytes/macrophages, as opposed to X-type histiocytoses in which the infiltrates contain Langerhans cells.
[2] [5] The skin can be affected with rashes, bumps and ulcers, and bones can be painful. There may be large lymph nodes and signs of lung and liver disease. Affected people may present with fever and weight loss. [1] The face may look asymmetrical and ear infections are common. [5]