J Korean Child Neurol Soc.  1998 May;5(2):346-350.

A Case of Epidermal Nevus Syndrome with Infantile Spasm

• Kim HS
• Shin YK
• Lee KH
• Eun BL
• Park SH
• Kim IH

Abstract

Epidermal nevus syndrome is a rare congenital malformation clinically characterized by the association of epidermal nevi with multiple abnormalities of the skin, skeletal system, central nervous system, eyes, cardiovascular system, urogenital system, as well as with malignant conditions. We describe a 4-month-old male with an extensive epidermal nevus involving the left side of the body (nevus unius lateris) and associated with lymphedema of the left lower extremity, diffuse alopecia and focal cicatrical alopecia of the scalp, atrial septal defect, multicystic dysplastic kidney and infantile spasm. We report this case with brief review of related literatures.

MeSH Terms

Abnormalities, Multiple
Alopecia
Cardiovascular System
Central Nervous System
Heart Septal Defects, Atrial
Humans
Infant
Infant, Newborn
Lower Extremity
Lymphedema
Male
Multicystic Dysplastic Kidney
Nevus*
Scalp
Skin
Spasms, Infantile*
Urogenital System