XANTHELASMA

Xanthelasma is a benign lipid deposition that occurs in middle-aged or elderly individuals. The lesions appear as multiple, soft, yellowish plaques, most commonly found in the inner aspects of the upper and lower eyelids. They usually begin as small lesions several millimeters in diameter, and may progress to larger growths several centimeters in the longest dimension (152).

Although the lesions often occur in patients with normal serum cholesterol levels, patients with xanthelasma may have an elevated serum cholesterol level. Diabetes and vascular disease may also be associated with xanthelasma.11, 19 The yellow plaques are made up of lipid-containing foam cells which appear in the superficial dermis.20

TREATMENT

Excision of the lesions with primary repair is the usual method of removing xanthelasma. In some cases, the lesion will be large enough that a complex repair with advancement flaps or grafts might be indicated. In these cases, the surgeon might excise part of the lesion, allow the wound to heal and the skin to stretch, and then excise the rest of the lesion at a later time.

The CO2 laser has been used with success to eradicate xanthelasma. This laser works by vaporizing the lesions. The noticeable color and texture difference between tumor and normal tissue permits an easy determination as to when the entire tumor has been vaporized. Use of the laser provides excellent hemostasis. Cosmetic results are good because defects created by laser vaporization do not extend deeper than the superficial reticular layer of the dermis, and these shallow defects heal without scarring. Xanthelasma is usually confined to this dermal layer.

AMYLOIDOSIS

Amyloid is a translucent glycoprotein which is deposited in many organs of the body in patients with amyloidosis, including the eyelids (153, 154). The lesions are typically papules which exhibit a yellow or waxy appearance. Purpura may be associated with these papules. Hemorrhagic papules involving the eyelids is very typical of primary systemic amyloidosis.22 Systemic disease may be manifested years after the eyelid involvement.23

NECROBIOTIC

XANTHOGRANULOMA

Necrobiotic xanthogranuloma with paraproteinemia is characterized by the development of multiple, painless, nonpruritic nodules or plaques, involving mainly the trunk and proximal part of the limbs, as well as the periorbital area. Lesions appear either as firm, superficial, waxy, yellowish plaques with prominent telangiectasia, as deep violaceous plaques, or as flesh-colored nodules. Lesions may ulcerate and undergo some degree of scarring. Associated systemic findings include dysproteinemia, cryoglobulinemia, leukopenia, or hyperlipemia. The nodules may also be associated with significant hematological disorders such as myeloma or other lymphoproliferative disorders.

Biopsy of the lesion often assists in making a diagnosis of necrobiotic xanthogranuloma. Systemic evaluation for associated diseases should then be performed. Treatment, which should be directed at the underlying disorder, may result in resolution of the nodules.24

NEUROFIBROMATOSIS (VON RECKLINGHAUSEN’S DISEASE)

Neurofibromatosis is a systemic condition characterized by diffuse proliferation of the Schwann cells of the peripheral nerves. It is one of the most common inherited disorders in humans with an estimated frequency of 1 per 3,000. About one-half of affected individuals clearly inherit the disease from the parent as an autosomal dominant trait. The other half appears to have a condition that results from new genetic mutations.25

The eyelids may show diffuse thickening and hypertrophy of the skin making the eyelids hang in baggy folds. A proliferation inside the nerve sheath may result in marked thickening and tortuosity of the nerves. This is referred to as a plexiform neurofibroma. The diffuse thickening and hypertrophy of the skin and/or the plexiform neurofibroma may result in an ‘S’-shaped configuration to the upper eyelid or severe ptosis (155, 156).

Small, multiple skin nodules occur on all parts of the body, including the eyelids. These may be pedunculated and are due to proliferation of fibrous tissue. The nodules are referred to as fibroma molluscum. Neurofibromas may occur in the bony orbit, the conjunctiva, the uvea, the retina, and the optic nerve, as well as any other parts of the body. Hyperpigmented macules, known as café-au-lait spots, are one of the typical features of this condition, and may be present on any skin surface from the first year of life.

TREATMENT

Treatment of the eyelid involvement is limited. The lesions do not respond to radiation therapy. Surgical treatment of ptosis is often temporary since the tumors continue to grow. In some instances, removal of a neurofibroma is necessary to relieve pain.

REFERENCES

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