A clinical and immunological study of Netherton's syndrome.

Abstract

Netherton's syndrome is a rare genodermatosis of unknown cause, which is classified as an ichthyosiform syndrome. A clinical and immunological study of seven patients with Netherton's syndrome illustrates the clinical spectrum of this disorder, the frequent association with atopy, and the absence of consistent immunological abnormalities. Failure to thrive in infancy was a feature in six of the seven patients, and was considered to be life-threatening in three. The skin disease evolved into ichthyosis linearis circumflexa in four of the seven, and the remaining three patients suffered from persistent or recurrent ichthyosiform erythroderma.