XP is approximately 3 times more common in women and does not appear to have any ethnic variation. Approximately half of patients with XP have a lipid disorder. While the majority of these cases occur in the setting of acquired hyperlipidemia, XP is also seen in the setting of type II hyperlipoproteinemia. XP has been shown to be an independent risk factor for atherosclerosis and, consequently, ischemic cardiovascular disease.
H02.60 – Xanthelasma of unspecified eye, unspecified eyelid
6400008 – Xanthoma of eyelid
- Necrobiotic xanthogranuloma – Periorbital plaques are extensive and commonly involve the medial canthus. Extrafacial plaques have active erythematous borders and often have central atrophy or ulceration.
- Generalized plane xanthomas – The face, neck, and trunk may be involved; may be associated with hyperlipidemia, paraproteinemia, myeloma, or other lymphoid malignancies.
- Adult-onset asthma and periocular xanthogranuloma – A non-Langerhans cell histiocytosis with bilateral yellowish periorbital plaques and deeper nodules often with associated edematous appearance. Some cases have been associated with IgG4-related disease.
- Erdheim-Chester disease – A non-Langerhans cell histiocytosis in which approximately 25% of patients will have xanthelasma-like lesions very similar to XP but usually more extensive laterally and not associated with the typical hyperlipoproteinemia seen in XP.
- Syringoma – Confluence of smooth papules; less yellow than XP and tends to favor the lower lid area.
- Palpebral sarcoid – Yellow-brown to purple papules and plaques progress to annular or circinate configurations, often with associated lid edema. See sarcoidosis.
- Lipoid proteinosis – Beaded papules at the margins of the eyelids.
- Basal cell carcinoma (BCC) – May occur on the eyelid or medial or lateral canthus. Usually solitary and typical BCC morphology.
- Sebaceous hyperplasia
- Other forms of localized plane xanthoma