2026 ICD-10-CM Diagnosis Code Q80.4Harlequin fetus

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q80

ICD-10-CM Q80.4
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q80.4 is a billable ICD-10-CM diagnosis code for harlequin fetus. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 606 through 607. The code is exempt from POA reporting. Coders also document this condition as congenital ectropion. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q80.4
Billable Status
Yes — Valid for Submission
Code Describes
Harlequin fetus
Short Description
Harlequin fetus
Same as the full description in the CMS dataset.
Parent Code
Q80
Congenital ichthyosis

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ80Congenital ichthyosis
This CodeQ80.4Harlequin fetus

Present on Admission (POA)Billing

Q80.4 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Congenital ectropion
  • Congenital ectropion of lip
  • Harlequin change
  • Harlequin fetus
  • Harlequin ichthyosis
  • Harlequin syndrome
  • Segmental hyperhidrosis

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Harlequin(newborn)
    • Ichthyosis(congenital)
      • fetalis

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL010
Other specified and unspecified congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Harlequin Ichthyosis

    a very rare and usually lethal autosomal recessive inherited disorder of the skin caused by mutations in the abca12 gene. it is characterized by the presence of hard and thick skin. there are diamond-like plates formed in the skin which are separated by fissures.

Patient EducationClinical

Skin Conditions

Your skin is your body's largest organ. It covers the entire outside of your body. There are many ways that your skin protects your body and helps keep you healthy. For example, it:

The full article covers:

  • What does your skin do?
  • What problems and conditions can affect your skin?
  • How can I keep my skin healthy?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q80.4 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
757.1 Ichthyosis congenita
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About Q80.4Overview

Is Q80.4 (Congenital ichthyosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report harlequin fetus on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q80.4 group to?

When harlequin fetus is the principal diagnosis on an inpatient stay, it groups to MS-DRG 606, 607, with relative weights from 0.9064 to 1.5132 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q80.4 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for harlequin fetus on inpatient claims.

What is the ICD-9 equivalent of Q80.4?

Under the General Equivalence Mappings, harlequin fetus converts to ICD-9-CM 757.1 (ichthyosis congenita). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.