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The most common skin lesion is the sebaceous adenoma, presenting as yellow papules or nodules predominantly on the face. Histologically sebaceous adenoma has a nodular lobulated growth with dark and light areas corresponding to generative cells (dark) and sebaceous cells (light) with cytoplasmic lipid vacuoles. Sebaceous epitheliomas differ in the degree of differentiation to adenomas and microscopically may appear similar to a basal cell carcinoma, but with focal sebaceous differentiation. Sebaceous carcinomas are typically found on the eyelids as yellow nodules with tendency to ulceration and are locally aggressive in nature. Excision and cryotherapy are helpful techniques for removal of the skin lesions. Isotetinoin and interferon-α2A are been suggested in treating early lesions and also preventing tumor development. Keratoacanthomas may appear in 20% of patients with typical histologic features except on the rare occasion when they may show sebaceous differentiation.