Epidermal Nevus Syndrome with Hypermelanosis and Chronic Hyponatremia
Resource
PEDIATRIC NEUROLOGY v.22 n.2 pp.151-154
Journal
PEDIATRIC NEUROLOGY
Journal Volume
v.22
Journal Issue
n.2
Pages
151-154
Date Issued
2000
Date
2000
Author(s)
YU, TSUI-WEN
TSAU, YONG-KWEI
YOUNG, CHAINLLIE
CHIU, HSIEN-CHING
SHEN, YU-ZEN
Abstract
Epidermal nevus syndrome is seldom encountered, and its association with hypermelanosis and the chronic syndrome of inappropriate antidiuretic hormone secretion (SIADH) has never been reported. A male neonate who developed intractable seizures and hyponatremia soon after birth is reported. He had alopecic patches on the scalp at birth. Large areas of skin hyperpigmentation, and epidermal nevi developed gradually. The clinical picture of hypotonic hyponatremia, high urine osmolality, elevated urine sodium, and euvolemia was compatible with SIADH. The seizures did not correlate with the hyponatremia, and no other cause for the seizures could be identified. The hyponatremia became chronic and was treated with a direct supply of sodium chloride. The development of the patient was markedly delayed at the last visit when he was 1 year of age. It is suggested that hypermelanosis and chronic SIADH may also be a variant presentation of epidermal nevus syndrome.
Type
journal article