Incontinentia pigmenti achromians (Ito)

Abstract

In 1951, Ito reported the first case of an abnormal, cutaneous, hypopigmented lesion and titled an incontinentia pigmenti achromians. Thereafter, 90 other cases have been reported including 33 cases in Japan. This neurocutaneous disorder is characterized by hypopigmented lesions that have a particular wild or streaked appearance and we have found a high incidence of associated abnormalities of the craniofacial region, the CNS and the muscoskeletal system. Among cases which we reviewed, 43 (48%) had associated craniofacial abnormalities and 11 (12%) had associated hand and foot abnormalities, however, we have only found one case report in the literature of plastic surgery. In general, we describe this lesion and its associated abnormalities bases on the 90 cases that have been reported.