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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 ...
Juvenile xanthogranuloma (JXG) II: D76.3: non-Langerhans-cell histiocytosis Hemophagocytic lymphohistiocytosis (HLH) II: D76.1: non-Langerhans-cell histiocytosis Niemann–Pick disease: II: E75.2: non-Langerhans-cell histiocytosis Sea-blue histiocytosis: II – non-Langerhans-cell histiocytosis Acute monocytic leukemia: III: C93.0: malignant ...
Juvenile xanthogranuloma: Touton type giant cells and foamy cells VI. Tumoral lesions: Benign tumoral lesions Mastocytoma: Abundant mast cells Tzanck smear test is useful for rapid diagnosis of mastocytoma in children Sebaceous hyperplasia: Clusters of sebocytes Seborrheic keratosis: Hyperkeratosis and horny cysts 87.5% sensitive and 80.8% specific
Juvenile xanthogranuloma; Progressive nodular histiocytoma; Necrobiotic xanthogranuloma (Giant Cell) Reticulohistiocytoma; Multicentric reticulohistiocytosis; Rosai–Dorfman disease; Xanthoma disseminatum; Kikuchi disease; Erdheim–Chester disease. [2]
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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.
Disseminated juvenile xanthogranuloma Erdheim–Chester disease Treatment. Treatment can occasionally consist of "watchful waiting" (e.g., in CLL ...