E70.331 ICD-10-CM Code: Hermansky-Pudlak syndrome
E70.331 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · HCC Buddy coding tools
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E70.331
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceHermansky-Pudlak syndrome
Hermansky-Pudlak syndrome is a rare inherited disorder that affects the body's ability to produce and store pigment, resulting in light skin, light-colored eyes, and bleeding problems due to abnormal blood platelets. Patients with this condition are at increased risk for lung disease and other complications.
Buddy Insight
Hermansky-Pudlak syndrome is a rare autosomal recessive disorder characterized by oculocutaneous albinism, a bleeding diathesis from platelet storage pool deficiency, and in several subtypes, progressive pulmonary fibrosis or granulomatous colitis.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
Context neededHCC 028
Coefficient needs member context
ESRD/PACE
Context neededHCC 23
Coefficient needs member context
RXHCC
MappedHCC 43
Code-level coefficient reference
Code Book Path
Inclusion Terms
OfficialNo inclusion terms are included in this display for E70.331. Check the code and parent instructions in the Code Book.
Excludes 2
Official- Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88
Related Codes
Includes
OfficialNo Includes notes are included in this display for E70.331. Check the code and parent instructions in the Code Book.
Excludes 1
Official- transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88
- androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88
- congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88
- hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88
- Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88
- 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88
Code First
OfficialNo Code First sequencing instructions are included in this display for E70.331. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for E70.331. Check the code and parent instructions in the Code Book.
Code Also
OfficialNo Code Also instructions are included in this display for E70.331. Check the code and parent instructions in the Code Book.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
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 E70.331 an HCC code?
E70.331 has no mapping under the current CMS-HCC V28 community payment model. E70.331 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E70.331 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping.
- Code
- E70.331
- Description
- Hermansky-Pudlak syndrome
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF reference coefficient
- —
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. 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 context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.
Work E70.331 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for E70.331
For E70.331, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.
- 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
Coder workflow notes
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What This Code Means
E70.331 is the ICD-10-CM diagnosis code for hermansky-pudlak syndrome. Hermansky-Pudlak syndrome is a rare inherited disorder that affects the body's ability to produce and store pigment, resulting in light skin, light-colored eyes, and bleeding problems due to abnormal blood platelets. Patients with this condition are at increased risk for lung disease and other complications. E70.331 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
E70.331 has no mapping under the current CMS-HCC V28 community payment model. E70.331 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E70.331 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.
This is a specific subtype code (E70.331) that indicates Hermansky-Pudlak syndrome type 1; verify the specific type documented in the medical record as other types (E70.332, E70.339) exist.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E70.331 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is a specific subtype code (E70.331) that indicates Hermansky-Pudlak syndrome type 1; verify the specific type documented in the medical record as other types (E70.332, E70.339) exist
- •This is a rare genetic disorder; ensure documentation clearly states the diagnosis and consider querying the provider if the condition is mentioned without specification of type, as accurate subtype coding is important for rare disease registries and research
Clinical Significance
Hermansky-Pudlak syndrome is a rare autosomal recessive disorder characterized by oculocutaneous albinism, a bleeding diathesis from platelet storage pool deficiency, and in several subtypes, progressive pulmonary fibrosis or granulomatous colitis. There are multiple genetic subtypes (HPS-1 through HPS-10), with HPS-1 and HPS-4 carrying the highest risk of fatal pulmonary fibrosis. This condition is particularly prevalent in Puerto Rican populations.
Documentation Requirements
- ✓Documentation should include genetic testing confirming the specific Hermansky-Pudlak syndrome subtype, platelet electron microscopy showing absent dense bodies, bleeding history and severity, pulmonary function testing and chest imaging for fibrosis surveillance, and gastrointestinal evaluation if colitis symptoms are present.
- ✓The specific subtype is important as it determines pulmonary fibrosis risk.
- ✓Ophthalmologic findings and visual acuity should be documented.
Commonly Confused Codes
- •E70.330 (Chediak-Higashi syndrome) combines albinism with immunodeficiency rather than pulmonary fibrosis.
- •E70.338 (Other albinism with hematologic abnormality) covers other combined syndromes.
- •J84.10 (Pulmonary fibrosis, unspecified) may be coded additionally for lung involvement.
- •K52.89 (Other specified noninfective gastroenteritis and colitis) may code the colitis component.
- •D69.1 (Qualitative platelet defects) captures the bleeding disorder.