Serum steroid sulphates in ichthyosis

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