[Necrobiotic xanthogranuloma with eye involvement. Overview and case report].

Necrobiotic xanthogranuloma is a rare disease marked by yellowish-brown plaques with destructive growth and a tendency to ulceration, which shows a characteristic histopathological pattern of palisading granuloma with foam cells, Touton's and foreign-body giant cells, bizarre multinuclear giant cells, cholesterol clefts and marked necrobiosis of the connective tissue. Necrobiotic xanthogranuloma is associated with IgG paraproteinaemia, and occasionally with an IgA paraproteinaemia. Ocular symptoms and visceral involvement are common. We report a case of a 53-year-old woman with periorbital necrobiotic xanthogranuloma, ocular involvement and IgG paraproteinaemia, who had undergone argon-laser treatment to no avail following a diagnosis of xanthelasma. Periorbital xanthelasma-like lesions with a tendency to ulceration in combination with unexplained ocular symptoms consisting of keratitis, scleritis, episcleritis or uveitis should suggest the diagnosis of necrobiotic xanthogranuloma, especially if they are associated with paraproteinaemia.