| Abstract: | ![]()
Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occursin all patients. Additionally, other ectodermal tissues may be affected, such as thecentral nervous system, eyes, hair, nails and teeth. The disease has a X-linkeddominant inheritance pattern and is usually lethal to male fetuses. Thedermatological findings occur in four successive phases, following the lines ofBlaschko: First phase - vesicles on an erythematous base; second phase - verrucoushyperkeratotic lesions; third phase - hyperchromic spots and fourth phase -hypochromic atrophic lesions. |
