INTRODUCTION Xanthogranulomas are lesions of lipid accumulation with foamy histiocytes. Different forms of xanthogranuloma are based on specific histologic findings and on clinical associations such as ocular lesions (juvenile xanthogranuloma), paraproteinemia and necrobiosis (necrobiotic xanthogranuloma), adult lesions with or without new onset asthma (adult onset xanthogranu-loma), and visceral deposits with bone lesions (Erdheim-Chester Disease). They occur most commonly on the face, scalp, and upper trunk, and whites are affected more than blacks. These lesions can be associated with elevated serum lipid levels but usually lipids are normal.

CLINICAL PRESENTATION Cutaneous lesions may be single or multiple, well-demarcated to diffuse, elevated, painless plaques that can occur unilaterally or bilaterally. They are yellowish in color with an orange peel-like surface. Lesions can be soft or more indurated and rubbery in consistency. Multiple lesions are more likely to have eye findings associated with juvenile xanthogranuloma. The eyelid lesions may extend into the anterior orbit by infiltration of muscles, lacrimal gland, and retrobulbar fat. With orbital involvement the patient may present with lid retraction, ptosis, chemosis, motility disturbance, proptosis, or an afferent papillary defect from extension along the optic nerve.

Erdheim Chester Eye
(Courtesy of Robert A. Goldberg, M.D.)

HISTOPATHOLOGY Xanthogranulomas in adults may resemble juvenile xanthogranulomas histologically with lipid-laden macrophages (foam cells) and Touton giant cells along with interspersed lymphocytes and plasma cells.

Differential Diagnosis For Lymphocytosis

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes juvenile xanthogranuloma, low grade necrobiotic xanthogranuloma, Erdheim-Chester disease, xanthelasma, dermatofibroma, lipoid proteinosis, tuberous xanthoma, lipoma, fibrous histiocytoma, lymphoma, neurofibroma, and amyloidosis.

TREATMENT While xanthogranulomas are benign they can be associated with significant morbidity and mortality when there is systemic involvement. Therefore all patients should have systemic evaluation to rule out Erdheim-Chester disease and necrobiotic xanthogranuloma. Surgical excision of the lesion will provide cosmetic improvement. Corticosteroid injections with triamcinolone acetonide have been effective and avoid the systemic complications of cytotoxic agents. Low dose periorbital radiotherapy is also an option. For resistant lesions cytotoxic agents may be required.

Essentials of Human Physiology

Essentials of Human Physiology

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