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ICD-10-CM 2027 diagnosis code

E71.540Rhizomelic chondrodysplasia punctata

✓ Billable / specificCC — complication/comorbidity

E71.540 is a valid, billable ICD-10-CM code for rhizomelic chondrodysplasia punctata. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.

Looking up by condition? See ICD-10 code for Rhizomelic chondrodysplasia punctata.

Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About E71.540

E71.540 is the ICD-10-CM diagnosis code for Rhizomelic chondrodysplasia punctata. It belongs to category E71 (disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E71.54 (other peroxisomal disorders), E71.540 is specifically for Rhizomelic chondrodysplasia punctata. Related codes cover Zellweger-like syndrome (E71.541), other group 3 peroxisomal disorders (E71.542) and other peroxisomal disorders (E71.548).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 277.86 (peroxisomal disorders).

Notes that apply from higher levels

Instructions written at a parent level also apply to E71.540.

› From E71.5 Peroxisomal disorders
Excludes1
  • Schilder's disease (G37.0)

Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.

Alphabetic index entries

2 entries

Terms in the official ICD-10-CM index that lead to E71.540.

Codes whose notes reference E71.540

MS-DRG v44.0 grouping

All DRGs →

Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.

MDC 10 · Endocrine, Nutritional and Metabolic Diseases and Disorders

ICD-9-CM equivalent

Converter →
  • 277.86Peroxisomal disordersapproximate

From the CMS 2018 General Equivalence Mappings (GEMs), the final GEMs release.

Code history

  1. 2016
  2. 2017
  3. 2018
  4. 2019
  5. 2020
  6. 2021
  7. 2022
  8. 2023
  9. 2024
  10. 2025
  11. 2026
  12. 2027
  • FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
  • No changes since FY2016.

Common questions about E71.540

Is E71.540 billable?

Yes. E71.540 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can E71.540 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict E71.540 as a principal diagnosis.

Is E71.540 a CC or MCC?

E71.540 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.

What DRG does E71.540 group to?

E71.540 is used in the MS-DRG v44.0 logic for MS-DRG 642 (Inborn and Other Disorders of Metabolism). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.

What is the ICD-9 code for E71.540?

The CMS General Equivalence Mappings map E71.540 to ICD-9-CM 277.86.