Serum steroid sulphates in ichthyosis

Abstract

Serum concentrations of pregnenolone sulfate, dehydroepiandrosterone sulfate and 5-androstene-3.beta.,17.beta.-diol sulfate were measured by radioimmunoassays in 12 patients with various types of ichthyosis. In X-linked ichthyosis (n = 5), ichthyosis vulgaris (n = 5) and lamellar ichthyosis (n = 1), steroid sulfates were not higher than in the control subjects. In 1 baby with ichthyosiform erythroderma and associated deafness serum 5-androstene-3.beta.,17.beta.-diol sulfate concentration was about 60-fold higher (31 .mu.g/ml) than the mean of the control children. The other steroid sulfate levels were normal in this baby. Apparently it is not possible to demonstrate the steroid sulfatase deficiency in X-linked ichthyosis by determining blood steroid sulfates. The cause of the high 5-androstene-3.beta.,17.beta.-diol sulfate concentration in the baby with ichthyosiform erythroderma needs further evaluation.