Necrobiotic xanthogranuloma is a rare disease marked by yellowish-brow
n plaques with destructive growth and a tendency to ulceration, which
shows a characteristics histopathological pattern of palisading granul
oma with foam cells, Touton's and foreign-body giant cells, bizarre mu
ltinuclear giant cells, cholesterol clefts and marked necrobiosis of t
he connective tissue. Necrobiotic xanthogranuloma is associated with I
gG paraproteinaemia, and occasionally with an IgA paraproteinaemia. Oc
ular symptoms and visceral involvement are common. We report a case of
a 53-year-old woman with periorbital necrobiotic xanthogranuloma, ocu
lar involvement and IgG paraproteinaemia, who had undergone argon-lase
r treatment to no avail following a diagnosis of xanthelasma. Periorbi
tal xanthelasma-like lesions with a tendency to ulceration in combinat
ion with unexplained ocular symptoms consisting of keratitis, scleriti
s, episcleritis or uveitis should suggest the diagnosis of necrobiotic
xanthogranuloma, especially if they are associated with paraproteinae
mia.