Xanthelasma is an extremely common, benign subcutaneous eyelid lesion that has characteristic clinical and histopathologic features (1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26). When it is larger and nodular, assuming tumorous proportions, it is called a xanthoma. Xanthelasma tends to be bilateral and more common in the elderly. It occurs in 1% to 3% of individuals and is slightly more common in women (4). Although some patients with xanthelasma are normolipemic, about 50% of them have essential hyperlipidemia (usually type II) or secondary hyperlipidemia owing to conditions like diabetes mellitus or biliary cirrhosis (1,2,3,4,5,6).

Tuberous xanthoma appears as solitary or multiple, placoid or papular lesions that have a predilection for buttocks, elbows, knees, and fingers. It is more likely to be associated with hyperlipidemia types II and III (1). It may be a result from hyperlipemic genetic defects in lipid metabolism, and is most often transmitted as an autosomal-dominant trait. It consists of one or more elevated nodules that are usually located in the extremities, but can be found on the eyelids (7,8,9,10,11). One case of multiple bilateral eyelid tumors was initially believed to represent fibrous histiocytomas (1), but later the diagnosis was changed to tuberous xanthomas (2). (See Figure 10.10.)

Multiple eruptive xanthomas can sometimes occur in patients who experience a rapid rise in serum triglyceride levels (5). In some cases, these lesions can become very large and aggressive despite being benign, and even invade through the orbital septum into the orbital fat. Interestingly, these large xanthomatous lesions have been known to extensively involve all four eyelids in patients with normal serum lipid levels (8,9,10,11).

Affected patients are believed to be at higher risk of death from cardiovascular disease (4). Xanthelasma is also seen with greater frequency in patients with the Erdheim-Chester disease, an idiopathic condition characterized by lipid deposition in bones, heart, retroperitoneum, and orbit (12,13,14).

Clinically, xanthelasma appears as one or more, flat or minimally elevated, yellow, placoid lesions that affect the loose skin of the eyelids, more commonly on the medial aspect of the eyelids. It generally occurs in middle-aged or older adults and is more common in women. It is often bilateral and symmetrical, and can sometimes slowly enlarge and coalesce to form large, raised, plaquelike, or nodular lesions. When a xanthelasma becomes elevated and nodular, it is more properly called a tuberous xanthoma (7,8,9,10,11).

Microscopically, xanthelasma and xanthoma are forms of lipoma that consist of an infiltration of the superficial reticular dermis by foamy histiocytes. The cells tend to be more concentrated around blood vessels (1,2,3,4,5). Touton giant cells are occasionally present. There is generally an absence of fibrosis.

There are several approaches to management of xanthelasma and xanthoma, depending on the clinical circumstances (15,16,17,18,19,20,21,22,23,24,25,26). Management should include evaluation of the affected patient for various hyperlipidemias and Erdheim-Chester disease. The eyelid lesions can generally be observed. Surgical excision should be considered for larger or cosmetically unacceptable lesions. The wound can be closed with sutures or allowed to heal by secondary intention (16). An intriguing technique has been reported in which the xanthelasma is raised with a skin flap and resected from the back side of the skin and then the skin flap without the tumor is sutured into its original position (19). Others have modified that technique to treat the xanthelasma on the back side of the flap with erbium yttrium aluminum garnet (YAG) laser and then suturing the flap into position (25). Topical application of 35% trichloroacetic acid can also be effective (5). Others have advocated topical 100% bichloracetic acid (18). Very good results have also been obtained by using a carbon dioxide laser to vaporize the lesions (15,21). More recently, the erbium YAG laser has also been reported to be effective (23). The treatment options have recently been reviewed in detail (24). In addition, affected patients should be treated medically for elevated serum lipids. Sometimes such medical treatment alone brings about resolution of xanthelasma (26). The larger, tuberous xanthomas generally require surgical excision.

1. Shields CL, Mashayekhi A, Racciato P, et al. Disappearance of eyelid xanthelasma following oral simvastatin (Zocor TM ).Br J Ophthalmol 2005;89:639.

2. Shields JA, Karcioglu Z, Shields CL, et al. Orbital and eyelid involvement with Erdheim-Chester disease. Arch Ophthalmol 1991;109:850-854.

Juvenile xanthogranuloma (JXG) is an idiopathic granulomatous inflammation that usually affects the skin of infants, but can occur in ocular tissues of adults as well as children (1,2,3,4,5,6,7,8,9,10,11,12,13,14). Hence, the word “juvenile” may not be always accurate. We use “juvenile xanthogranuloma” here because of its widespread usage, but we briefly mention adult xanthogranuloma as well. In a series of 53 cases of ocular JXG, the eyelid was involved in 13 (25%; 1).