EPIDERMAL NEVUS SYNDROME - ASSOCIATION WITH CENTRAL PRECOCIOUS PUBERTY AND WOOLLY HAIR NEVUS

The epidermal nevus syndrome is characterized by the association of ep idermal nevi with abnormalities of the skin, skeletal system, central nervous system, eyes, and cardiovascular system, as well as with malig nant conditions. We describe a 2-year-old girl with an extensive epide rmal nevus involving the left side of the body (nevus unius lateris) a nd associated with a woolly hair nevus on the left parietal area of th e scalp and multiple acquired melanocytic nevi. Idiopathic central pre cocious puberty characterized by premature breast and pubic hair devel opment and advanced bone age developed at the age of 20 months. A shar p increase in serum gonadotropins after a luteinizing hormone releasin g hormone (LHRH) stimulation test confirmed the presence of central pr ecocious puberty. This is the third reported case of precocious pubert y associated with the epidermal nevus syndrome.