The correct answer is nevus sebaceous (choice “b”), a rather common hamartomatous congenital tumor. The diagnosis was confirmed by pathologic examination of a tiny sample from the most papular portion of the lesion.
Given the lesion’s congenital nature and complete lack of response to treatment, wart (choice “a”) was quite unlikely. And although trichofolliculoma (choice “c”) and epidermal nevus (choice “d”) were possible differential items, the former usually appears much later in life and the latter is usually more dry and rough to the touch.
Nevus sebaceous (NS) of Jadassohn was first described in 1895 by a Swedish dermatologist who had seen a series of young patients with hairless plaques in the scalp or on surrounding neck or facial skin. Pathologic exam confirmed them to be organoid nevi representing an overgrowth of sebaceous glands.
Over time, it became clear that NS affects all genders and races equally. For most patients, the lesions are of cosmetic concern due to the lack of hair. But it has also been established that NS can develop in areas, including the face, ears, and neck, on which they may be cosmetically significant and difficult to remove.
Concern arose when reports began to surface that NS could undergo malignant transformation, especially in larger scalp lesions that are subject to years of excess UV exposure. This was the driving force behind the common practice of removing NS at puberty. We now know that although basal or squamous cell carcinoma, or even melanoma, can develop in longstanding NS, the frequency is probably far less than previously thought.
Most cases of NS in the scalp are easy to diagnose by their pink color, plaquish morphology, and mammillated hairless surface (coupled with congenital manifestation). But a few, such as this patient’s ear lesion, require biopsy for confirmation. As this patient ages, he may feel the need to have the rest of it surgically removed.