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.

Adult-onset asthma with periocular xanthogranuloma (AAPOX), a variant of periorbital xanthogranulomatous disease: An uncommon entity

“…[ 1 ] APOX remains an uncommon entity and till now fewer than 50 cases reported in the English literature. [ 2 3 4 5 6 ] AAPOX is characterized clinically by periocular swelling with yellowish nodules and asthma. Patients generally have systemic lymphadenopathy, salivary gland enlargement, preseptal, and anterior orbital involvement, immune dysfunction, paraproteinemia and elevated IgG serum levels.…”

The clinical spectrum of xanthomatous lesions of the eyelids

“…However, there are intermediate cases presenting with only periorbital xanthogranulomas and/or also with mild hematological abnormalities that cannot be surely classified . Follow‐up of these patients in terms of possible development of systemic findings is recommended …”

“…21,39 Follow-up of these patients in terms of possible development of systemic findings is recommended. 40 Adult-onset xanthogranuloma Adult-onset xanthogranuloma usually presents as a unilateral, solitary, yellow-orange, elevated xanthomatous lesion. Eyelid lesions tend to be indurated and deeper than XP.…”

Adult Onset Orbital Xanthogranuloma, a Variant of Adult Orbital Xanthogranulomatous Disease: An Infrequent Entity

“…Xanthogranulomatous disorders of the orbit are a group of rare disorders of unknown etiopathogenesis that are a form of non-Langerhans cell (LC) histiocytosis. [ 1 ] They affect the skin and subcutaneous tissue of the orbit and ocular adnexae. [ 2 ] The disorders of this group show considerable heterogeneity and overlap, with some patients manifesting with only indolent disease localized to the skin, whereas others manifest with severe symptoms and systemic invasive disease.…”