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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
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]
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 ...
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).
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
The xanthogranulomatous type of inflammation is most-commonly seen in pyelonephritis and cholecystitis, although it has more recently been described in an array of other locations including bronchi, lung, endometrium, vagina, fallopian tubes, ovary, testis, epididymis, stomach, colon, ileum, pancreas, bone, lymph nodes, bladder, adrenal gland, abdomen and muscle. [5]
The other key term in the above definition is the word "organized" which refers to a tight, ball-like formation. The macrophages in these formations are typically so tightly clustered that the borders of individual cells are difficult to appreciate. Loosely dispersed macrophages are not considered to be granulomas.