ICD-10 Code E75.26 – Sulfatase deficiency (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Sulfatase deficiency is E75.26.
2026 ICD-10-CM Diagnosis Code E75.26 – Sulfatase deficiency

What it is

E75.26 identifies sulfatase deficiency, a rare inherited metabolic disorder caused by reduced activity of sulfatase enzymes. This leads to buildup of certain sulfate-containing substances in the body.

Clinical signs

Clinical features vary; refer to documentation. Common findings may include developmental delay, skeletal abnormalities, and progressive neurologic or physical impairment consistent with a lysosomal storage disorder.

When to use this code

Use E75.26 when the provider documents sulfatase deficiency or a confirmed sulfatase enzyme disorder. Apply it for inherited metabolic disease cases supported by clinical evaluation, genetic testing, or specialist assessment. If the record names a more specific subtype, code that diagnosis instead.

Do not use for

Do not use this code for isolated low sulfatase activity without a documented diagnosis. Check documentation if the condition is only suspected, ruled out, or described under a different metabolic disorder.

Coding tip

Verify the exact wording in the assessment; if the note names a specific sulfatase-related syndrome, code the most specific documented condition.

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