[3] This enzyme catalyses the following chemical reaction Also acts on some related steryl sulfates.

[3] A congenital deficiency in the enzyme is associated with X-linked ichthyosis, a scaly-skin disease affecting roughly 1 in every 2,000 to 6,000 males.

[7][8] The excessive skin scaling and hyperkeratosis is caused by a lack of breakdown and thus accumulation of cholesterol sulfate, a steroid that stabilizes cell membranes and adds cohesion, in the outer layers of the skin.

[11] Blood-clotting abnormalities may occur more frequently in males with XLI and female carriers.

[15][16] The most potent inhibitors are based around the aryl sulfamate pharmacophore[17] and it is thought that such compounds irreversibly modify the active site formylglycine residue of steroid sulfatase.