Subungual tumors in incontinentia pigmenti

A 22-year-old woman presented with intermittently painful subungual keratotic tumors of the hands as a late manifestation of incontinentia pigmenti. Scalloped bony deformities of the distal phalanges of both hands were noted on roentgenograms. The origin of these lytic deformities is uncertain; they may be secondary pressure phenomena or, alternatively, they may be an intrinsic manifestation of incontinentia pigmenti. These subungual tumors clinically resemble verrucae, keratoacanthomas, squamous cell carcinomas, or subungual fibromas. Therefore, it is important to recognize that subungual keratotic tumors may be a late manifestation of incontinentia pigmenti.