Incontinentia pigmenti

IP is a distinct neurocutaneous syndrome with a high incidence of systemic findings. It should be suspected in patients who present with characteristic cutaneous stigmata. However, skin markers may be subtle in older children and adults. Evaluation of patients with IP requires a meticulous search for cutaneous and extracutaneous manifestations of the syndrome. Management should focus on genetic counseling and potentially serious complications involving the eyes, teeth, nad CNS.