AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2018 Issue 4; New/Revised ICD-10-CM Codes
Multiple Sulfatase Deficiency
Code E75.26, Sulfatase deficiency, was created for multiple sulfatase deficiency (MSD), a rare inherited metabolic disorder that is caused by the deficiency of the formylglycine-generating enzyme (FGE) that is responsible for activating various sulfatase enzymes. Deficiency in the FGE results in defective functioning of sulfatase enzymes. The impairment causes signs and symptoms in the brain, skeleton and skin. While there is a wide spectrum in the effects of MSD, the age of onset distinguishes the three types of MSD. The most severe form, neonatal MSD, develops soon after birth. There may be loss of motor skills, epilepsy, spasticity...
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Article Overview
This article provides a concise overview of multiple sulfatase deficiency, including its underlying enzyme-related cause, major clinical features, and the age-of-onset patterns used to distinguish the condition’s forms. It is relevant to coders, CDI specialists, and clinicians who need a high-level understanding of the diagnosis and the associated ICD-10-CM classification.
Why This Topic Matters
Accurate identification of this rare disorder supports consistent diagnosis coding and better communication across clinical, genetic, and revenue cycle workflows.
What You Will Learn
- What multiple sulfatase deficiency is
- How the disorder is described at a high level in clinical terms
- How age of onset relates to the condition’s broad clinical patterns
- Why the diagnosis is associated with a specific ICD-10-CM code
Who Should Read This
- Medical coders
- Coding auditors
- Clinical documentation integrity specialists
- Physicians
- Geneticists
- Revenue cycle professionals
Codes Discussed
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