Adult orbital xanthogranulomatous disease: adult-onset xanthogranuloma of periorbital location

Abstract

Adult orbital xanthogranulomatous disease (AOXGD) is a rare granulomatous disorder, which has four subtypes: adult-onset xanthogranuloma (AOX), adult-onset asthma with periocular xanthogranuloma, necrobiotic xanthogranuloma and Erdheim-Chester disease. We report a 42-year-old woman who presented with yellowish nonulcerative nodules on her eyelids. On histopathological examination of a nodule, mild degeneration of collagen fibres was seen, with surrounding infiltration of numerous foam cells and Touton giant cells in the deep dermis. Lymphoid follicles were seen in the reticular dermis. There was no apparent necrobiosis of collagen fibres. There were no clinical symptoms of asthma and no laboratory signs of paraproteinaemia during a follow-up of more than 5 years. We diagnosed this case as AOX, but further long-term follow-up would be required for the differentiation from the other AOXGDs. Dermatologists should be aware of these rare granulomatous disease conditions with ocular/orbital location, because they may cause ophthalmological complications.