Korean J Dermatol.
1984 Jun;22(3):354-359.
A Case of Epidermal Nevus Syndrome
Abstract
- Epidermal nevus syndrome is now delineated as a congenitally acquired syndrome consisting of deformities of the skin (various linear epidermal or organoid nevi), central nervous system, skeleton, cardiovascular system, and renal system. A 4-year-old girl had linear epidermal nevus on the right lower extremity, right ear lobe and anterior neck, and multiple skeletal abnorrnalities since birth. And she had an adult fist-sied, soft, nontender mass on the right lower abdomen since 2 years of age. Radiologic examination showed scoliosis of the thoracic spines, deformities of the lumbar spines, focal gigantisms of the left hand and right foot, and other skeletal abnormalities. Histological examination of the linear, verrucous lesion of the right leg showed hyperkeratosis and papillomatosis. The surgically removed abdominal mass revealed the finding of hydrosalpinx.
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