Ichthyosis Hystrix Gravior (Porcupine Man; Lambert type
Ichthyosis): Part of a group of epidermal nevus syndrome characterized by
distinctive nevi on the skin apparent at birth that form in a line and
usually are excessively pigmented. Often associated with neurologic and
skeletal abnormalities (e.g., excessive bone growth, scoliosis, and
clubfeet). Mental retardation, brain atrophy, brain cysts, seizures, and
eye abnormalities may occur. Transmission is suggested to be an autosomal
dominant trait that occurs in approximately two thirds of cases. Singular or
multiple forms of the five major types of epidermal nevi have been
described: raised wart-like streaks, polyp-like masses forming in lines,
dark velvety spots, scaly streaks, or an orange, hairless, velvety patch
covering part of the face, nose, eyes and scalp.