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Serum steroid sulphates in ichthyosis.

A Ruokonen, A Oikarinen, R Palatsi

The British Journal of Dermatology

|September 1, 1980

Summary

Steroid sulphate levels in patients with ichthyosis were measured. Most patients showed normal levels, but one infant with ichthyosiform erythroderma had significantly elevated 5-androstene-3 beta,17 beta-diol sulphate.

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Area of Science:

Biochemistry
Dermatology
Genetics

Background:

Ichthyosis encompasses a group of genetic skin disorders characterized by dry, scaling skin.
Steroid sulphates are important metabolites involved in various physiological processes.

Purpose of the Study:

To investigate serum concentrations of specific steroid sulphates in patients with different types of ichthyosis.
To determine if steroid sulphate levels can serve as biomarkers for ichthyosis subtypes, particularly X-linked ichthyosis.

Main Methods:

Radioimmunoassays were employed to measure serum levels of pregnenolone sulphate, dehydroepiandrosterone sulphate, and 5-androstene-3 beta,17 beta-diol sulphate.
Measurements were taken in twelve patients diagnosed with various ichthyosis forms and compared to control subjects.

Main Results:

Serum steroid sulphate levels were not significantly elevated in patients with X-linked ichthyosis, ichthyosis vulgaris, or lamellar ichthyosis compared to controls.
One infant with ichthyosiform erythroderma and deafness exhibited a 60-fold increase in 5-androstene-3 beta,17 beta-diol sulphate, while other steroid sulphates remained normal.

Conclusions:

Determining blood steroid sulphates is not a reliable method for diagnosing steroid sulphatase deficiency in X-linked ichthyosis.
The elevated 5-androstene-3 beta,17 beta-diol sulphate in the infant with ichthyosiform erythroderma warrants further investigation to understand its underlying cause.

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