2027 ICD-10-CM Diagnosis Code E75.242Niemann-Pick disease type C
ICD-10-CM Codes›E00–E89›E70-E88›E75
- Billable — Valid for Submission
- Chronic Condition
E75.242 is a billable ICD-10-CM diagnosis code for Niemann-Pick disease type C. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. Coders also document this condition as dystonia due to Niemann-Pick disease type C. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
E75.242 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 23 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23, ESRD (V21) category 23, ESRD (V24) category 23, and RxHCC Part D (V08) category 41 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E75.242 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Dystonia due to Niemann-Pick disease type C
- Lipoidosis
- Niemann-Pick disease type C1
- Niemann-Pick disease type C2
- Niemann-Pick disease, type C
- Sphingomyelin/cholesterol lipidosis
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E75.242: its own notes plus those printed at E75.2, E75, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- adrenoleukodystrophy [Addison-Schilder] (E71.528)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E75.242, its category, or a range that includes it.
Excludes1 3
These codes carry an Excludes1 note naming E75.242: they are not reported together with it, unless the two conditions are unrelated.
- E78.7 Disorders of bile acid and cholesterol metabolismNiemann-Pick disease type C (E75.242)
- R79 Other abnormal findings of blood chemistryspecific findings indicating disorder of lipid metabolism (E75.-) names E75.-, which includes this code
- E78 Disorders of lipoprotein metabolism and other lipidemias
Code First 2
These codes say to code first a condition named in the note, and E75.242 is one of the conditions named.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Niemann-Pick Disease, Type C
an autosomal recessive lipid storage disorder that is characterized by accumulation of cholesterol and sphingomyelins in cells of the viscera and the central nervous system. type c (or c1) and type d are allelic disorders caused by mutation of the npc1 gene, which encodes a protein that mediates intracellular cholesterol transport from lysosomes. clinical signs include hepatosplenomegaly and chronic neurological symptoms. type d is a variant in people with a nova scotia ancestry.
Patient EducationClinical
Genetic Brain Disorders
A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E75.242 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E75.242Overview
What is the ICD-10 code for Niemann-Pick disease type C?
The ICD-10-CM code for Niemann-Pick disease type C is E75.242 (sometimes written as E75242). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E75.242 (Niemann-Pick disease) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report Niemann-Pick disease type C on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E75.242 group to?
When Niemann-Pick disease type C is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can E75.242 be reported with E71.528?
Not as a rule. The Excludes1 note at E75.2, which applies to E75.242, lists "adrenoleukodystrophy [Addison-Schilder] (E71.528)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What is the ICD-9 equivalent of E75.242?
Under the General Equivalence Mappings, Niemann-Pick disease type C converts to ICD-9-CM 272.7 (lipidoses). The mapping is approximate, so confirm the match fits the documentation.
Does E75.242 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. E75.242 mapped to HCC 23 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the PACE (CMS-HCC V22) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V21) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V24) category 23 (Other Significant Endocrine and Metabolic Disorders), and RxHCC Part D (V08) category 41 (Lysosomal Storage Disorders).
