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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
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).
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]
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 ...
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 ...
Areas of necrobiosis are often more extensive and less well defined than in granuloma annulare. Presence of lipid in necrobiotic areas may be demonstrated by Sudan stains. Cholesterol clefts, fibrin, and mucin may also be present in areas of necrobiosis. Depending on the severity of the necrobiosis, certain cell types may be more predominant.
Generalized granuloma annulare is a chronic granulomatous dermatosis that typically affects young adults' distal extremities and manifests as annular grouped papules. [2] ...