The analysis of 62 cases of nevus sebaceus of Jadassohn and the study of the literature allows to define the clinical and histo-pathologic features of this benign tumor. The massive development of sebaceous glands, papillomatous epidermal hyperplasia, and maturation of apocrines glands, are present in a considerable number of cases. The lesions occurred on the scalp, face, auricular area and neck and are usually present at birth as a yellowish orange area. At puberty, the lesion becomes yellow to dark-brown and is verrucoid and micronodular. Almost all are hair-deficit nevi, and the final stage is the development of secondary tumors of the skin within the lesion. The risk of degeneration in basal-cell carcinoma justifies systematic surgical treatment.

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