phakomatosis pigmentokeratotica phakomatosis pigmentokeratotica phakomatosis pigmentokeratotica is a rare neurocutanous condition characterized by the combination of an organoid sebaceous nevus and speckled lentiginous nevus.: 634–5 : 776 it is an unusual variant of epidermal naevus syndrome. it was first described by happle et al. it is often associated with neurological or skeletal anomalies such as hemiatrophy, dysaesthesia and hyperhidrosis in a segmental pattern, mild mental retardation, seizures, deafness, ptosis and strabismus. phakomatosis pigmentokeratoticaother namesorganoid nevus with sebaceous differentiation, a speckled-lentiginous nevus, and other associated anomalies specialtydermatology