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Necrobiotic xanthogranuloma, also known as necrobiotic xanthogranuloma with paraproteinemia, [1] is a multisystem disease that affects older adults, and is characterized by prominent skin findings. [ 2 ] : 707
Juvenile xanthogranuloma Necrobiotic xanthogranuloma Giant cell reticulohistiocytoma Multicentric reticulohistiocytosis Xanthoma disseminatum Rosai–Dorfman disease Atypical fibroxanthoma Melanoma: CD79a: Primary cutaneous marginal zone lymphoma Primary cutaneous follicular lymphoma Diffuse large B-cell lymphoma leg type: CD117: Mast cell ...
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).
Palisading granuloma and necrobiotic materials Foreign-body granuloma: Foreign body 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
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 ...
Necrobiotic disorders are characterized by presence of necrobiotic granuloma on histopathology. Necrobiotic granuloma is described as aggregation of histiocytes around a central area of altered collagen and elastic fibers. Such a granuloma is typically arranged in a palisaded pattern. [2]
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 ...
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 ...