A spitz nevus is a distinct type of melanocytic neoplasm that most often develops in children. Spitz nevi are benign neoplasms, but their management has historically been controversial because of their histologic resemblance to melanoma.

Formerly referred to as “benign juvenile melanoma,” and “Spitz juvenile melanoma,” these terms are no longer used as they are misleading.

Classic Spitz nevi present as a pink- or flesh-colored, dome-shaped papule or nodule that appears most frequently on the head/neck region or lower extremities, and is most often noted in childhood or adolescence (Fig. 8.6).

A pigmented Spitz nevus appears as a smooth dark brown to black papule (Fig. 8.7).

Lesions typically grow rapidly over 3 to 6 months then stabilize.

They are usually less than 6 mm but can vary in size from 2 mm to >2 cm.

Most often are well circumscribed with a smooth surface; telangiectasias may be present.

Multiple Spitz nevi arising within a nevus spilus has been described.

Clinical features and the use of dermoscopy can help identify Spitz nevi on physical examination.

Histopathologic features of classic Spitz nevi include large spindle and/or epithelioid cells, usually in the paucity or absence of melanin, the so-called Kamino bodies, and uniform architecture and absence of mitoses.