What is ICD-10 code E75.26?

E75.26 is the ICD-10-CM diagnosis code for Sulfatase deficiency. A rare inherited metabolic disorder caused by deficiency of sulfatase enzymes, leading to accumulation of sulfated compounds in the body and progressive neurological damage. It belongs to the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89). E75.26 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E75.26 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. E75.26 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. E75.26 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

Which HCC does E75.26 map to under V28?

E75.26 does not map to a CMS-HCC V28 payment category. Check the current CMS-HCC V28 model file for payment year 2026 rather than an older V24 tip sheet.

What does E75.26 pay in RAF?

E75.26 has no separately stated V28 community RAF weight. If it is not a V28 payment HCC, it adds nothing to a community RAF score.

What is the V28 vs V24 difference for E75.26?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. E75.26 was a payment HCC under V24 (Dementia Without Complication) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

What documentation supports E75.26?

Verify the specific type of sulfatase deficiency (arylsulfatase A, B, or C) in the medical record for accurate coding This code requires documentation of clinical manifestations such as neurological symptoms or organ involvement Because E75.26 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score. See the MEAT criteria checklist at /meat-criteria for what Monitor, Evaluate, Assess, or Treat looks like in a note. MEAT is generic documentation evidence, not a payer-specific rule.

2026 ICD-10-CM E75.26: Sulfatase deficiency

E75.26 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC Buddy coding tools

ICD-10-CM Code View

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Code lookupE75.26Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E75.26

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Sulfatase deficiency

A rare inherited metabolic disorder caused by deficiency of sulfatase enzymes, leading to accumulation of sulfated compounds in the body and progressive neurological damage.

Buddy Insight

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Mapped

HCC 52

Dementia Without Complication

RAF 0.346

ACA/HHS

Mapped

HCC 119

HHS-HCC 119

Varies by metal level

ESRD/PACE

Mapped

HCC 52

Dementia Without Complication

RAF 0.042

RXHCC

Mapped

HCC 41

Lysosomal Storage Disorders

RAF 3.081

Code Book Path

Official

E75Disorders of sphingolipid metabolism and other lipid storage disorders

E75.2Other sphingolipidosis

E75.26Sulfatase deficiency

Inclusion Terms

Official

Multiple sulfatase deficiency (MSD)

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.26 in this effective period.

Related Child Codes

Official

E75.21Fabry (-Anderson) disease

E75.22Gaucher disease

E75.23Krabbe disease

E75.24Niemann-Pick disease

E75.25Metachromatic leukodystrophy

Includes

Official

ICD-10-CM does not list Includes notes for E75.26 in this effective period.

Excludes 1

Official

adrenoleukodystrophy [Addison-Schilder] (E71.528)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E75.26 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.26 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.26 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Confirmed diagnosis of multiple sulfatase deficiency or Austin disease

Enzyme assays showing deficiency of multiple sulfatases (arylsulfatase A, B, and C)

SUMF1 gene mutation analysis confirming formylglycine-generating enzyme deficiency

Documentation of clinical features from multiple pathways: leukodystrophy, skeletal changes, ichthyosis

MEAT Support

HCC Buddy guidance

Confirmed diagnosis of multiple sulfatase deficiency or Austin disease

Enzyme assays showing deficiency of multiple sulfatases (arylsulfatase A, B, and C)

SUMF1 gene mutation analysis confirming formylglycine-generating enzyme deficiency

Documentation of clinical features from multiple pathways: leukodystrophy, skeletal changes, ichthyosis

Audit Caution

HCC Buddy guidance

Confusing multiple sulfatase deficiency with isolated arylsulfatase A deficiency (metachromatic leukodystrophy)

Not recognizing that ichthyosis combined with leukodystrophy and skeletal changes suggests multiple sulfatase deficiency

Coding individual components (ichthyosis, leukodystrophy, skeletal changes) without identifying the unifying diagnosis

Using an isolated sulfatase deficiency code when the documentation supports multiple enzyme involvement

Common Mistakes

HCC Buddy guidance

E75.25 — Metachromatic leukodystrophy: involves only arylsulfatase A deficiency

E76.0-E76.29 — Mucopolysaccharidosis codes: MSD has MPS-like features but is a distinct condition

E75.23 — Krabbe disease: different enzyme entirely (galactosylceramidase)

E75.21 — Fabry disease: different lysosomal storage disorder

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is E75.26 an HCC code?

Yes. E75.26 maps to Dementia Without Complication under the V24 model but is not retained in V28.

Code: E75.26
Description: Sulfatase deficiency
HCC (V28): No CMS-HCC V28 mapping
RAF: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

V24HCC 52, Dementia Without Complication

0.346

ESRDHCC 52, Dementia Without Complication

0.042

RxHCCHCC 41, Lysosomal Storage Disorders

3.081

Each model's RAF is its CMS base weight for that model's standard population, so weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

Work E75.26 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E75.26

For E75.26 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
EEvaluate: test results, medication response, or physical findings reviewed by the provider
AAssess: explicit mention in the assessment or plan with acknowledgment of status
TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E75.26 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E75.26 is the ICD-10-CM diagnosis code for sulfatase deficiency. A rare inherited metabolic disorder caused by deficiency of sulfatase enzymes, leading to accumulation of sulfated compounds in the body and progressive neurological damage. E75.26 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the older CMS-HCC V24 model, E75.26 maps to Dementia Without Complication (HCC 52) with a community, non-dual, aged base RAF weight of 0.346. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

Verify the specific type of sulfatase deficiency (arylsulfatase A, B, or C) in the medical record for accurate coding. Because E75.26 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E75.26 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Verify the specific type of sulfatase deficiency (arylsulfatase A, B, or C) in the medical record for accurate coding
•This code requires documentation of clinical manifestations such as neurological symptoms or organ involvement

Clinical Significance

Sulfatase deficiency, also known as multiple sulfatase deficiency or Austin disease, is an ultra-rare condition where multiple sulfatase enzymes are simultaneously deficient due to a defect in the enzyme that activates all sulfatases. It combines features of metachromatic leukodystrophy, mucopolysaccharidoses, and other sulfatase deficiency disorders. The prognosis is severe with progressive neurological deterioration.

Documentation Requirements

✓Confirmed diagnosis of multiple sulfatase deficiency or Austin disease
✓Enzyme assays showing deficiency of multiple sulfatases (arylsulfatase A, B, and C)
✓SUMF1 gene mutation analysis confirming formylglycine-generating enzyme deficiency
✓Documentation of clinical features from multiple pathways: leukodystrophy, skeletal changes, ichthyosis
✓Neurological assessment documenting developmental status and regression
✓Current supportive care plan

Commonly Confused Codes

•E75.25: Metachromatic leukodystrophy: involves only arylsulfatase A deficiency
•E76.0-E76.29: Mucopolysaccharidosis codes: MSD has MPS-like features but is a distinct condition
•E75.23: Krabbe disease: different enzyme entirely (galactosylceramidase)
•E75.21: Fabry disease: different lysosomal storage disorder
•Q80.0: Ichthyosis vulgaris: ichthyosis in MSD is from sulfatase deficiency, not isolated