Case: The parents of this 6-year-old girl expressed concern about a yellow-brown arciform plaque on their daughter's cheek that has been present since birth. Although it has remained stable throughout her early childhood, her parents wonder whether it may become cancerous later on.
Would you recommend removing the lesion?
(Answer on next page)
Answer:
The lesion on this child's cheek is a nevus sebaceus of Jadassohn. These organoid nevi are present at birth and typically occur on the scalp; they often present in an arciform pattern that follows the skin lines of Blaschko. The lesions are usually subtle at birth, most commonly presenting as a slightly yellowish discoloration of the scalp in an area of alopecia (Figure). They become much more verrucous during adolescence.
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Nevus sebaceus of Jadassohn is a hamartoma that involves cells derived from sebaceous, follicular, and apocrine structures. These nevi are notorious for the development of benign secondary tumors related to the aforementioned cell types. It is often said that basal cell carcinomas develop in 10% of nevi sebaceus of Jadassohn; however, recent data suggest that the incidence is more likely 1%.1 The development of a sebaceous or apocrine carcinoma is a very rare event.
Therefore, the removal of these lesions is justified because of their location and their cosmetic appearance rather than their malignant potential. It is best to consider removal before their verrucous transformation. Because the nevi are derived from follicular, sebaceous, and apocrine cells, they extend into the deeper levels of the dermis; thus, any attempt to remove them by surface destruction will be unsatisfactory because of the resultant scarring. The current treatment of choice is excision—provided it will be cosmetically acceptable. The decision of whether to remove the lesion in the girl pictured is a difficult one; I recommend consultation with a plastic surgeon while she is still young.