G723 Necrobiotic Xanthogranuloma

Necrobiotic xanthogranuloma is a rare chronic and often progressive disorder with a predilection for the periorbital skin. Other areas of the face, as well as the trunk and limbs, can also be involved. Lesions present as sharply demarcated violaceus, partly xanthomatous nodules and plaques. Ulceration may develop. Almost all patients with necrobiotic xanthogranuloma are diagnosed with a paraproteinaemia. Other, more rare associations are hyperlipidaemia and leukopenia. Scleri-tis, episcleritis and keratitis are common ophthalmic complications. The histological changes are present in the dermis and in the subcutis. Large zones of necrobiotic collagen with hyaline and sometimes mucinous changes are present in the deep dermis. These areas are surrounded by histiocytes, partly with a foamy cytoplasm. Sometimes the xanthomatous changes are only minor. Multinucleated giant cells are easily found; they can be of the Touton type, but also of the foreign body type with bizarre nuclei. In ulcerating lesions, transepidermal elimination of debris can be seen.

Fig. 10.29. Xanthelasmata: multiple foamy histiocytes are present in the dermis

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