A CLINICAL AND IMMUNOLOGICAL STUDY OF NETHERTONS SYNDROME

Netherton's syndrome is a rare genodermatosis of unknown cause, which is classified as an ichthyosiform syndrome. A clinical and immunologic al study of seven patients with Netherton's syndrome illustrates the c linical spectrum of this disorder, the frequent association with atopy , and the absence of consistent immunological abnormalities. Failure t o thrive in infancy was a feature in six of the seven patients, and wa s considered to be life-threatening in three. The skin disease evolved into ichthyosis linearis circumflexa in four of the seven, and the re maining three patients suffered from persistent or recurrent ichthyosi form erythroderma.