2026 ICD-10-CM Diagnosis Code Q81.1Epidermolysis bullosa letalis

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q81

ICD-10-CM Q81.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q81.1 is a billable ICD-10-CM diagnosis code for epidermolysis bullosa letalis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 595 through 596. The code is exempt from POA reporting. Coders also document this condition as generalized junctional epidermolysis bullosa. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q81.1
Billable Status
Yes — Valid for Submission
Code Describes
Epidermolysis bullosa letalis
Short Description
Epidermolysis bullosa letalis
Same as the full description in the CMS dataset.
Parent Code
Q81
Epidermolysis bullosa

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ81Epidermolysis bullosa
This CodeQ81.1Epidermolysis bullosa letalis

Present on Admission (POA)Billing

Q81.1 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.

  • Generalized junctional epidermolysis bullosa
  • Junctional epidermolysis bullosa gravis of Herlitz

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Herlitz' syndrome

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.

    • Epidermolysis
      • bullosa (congenital)
        • letalis
    • Herlitz' syndrome

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

  • Epidermolysis Bullosa

    group of genetically determined disorders characterized by the blistering of skin and mucosae. there are four major forms: acquired, simple, junctional, and dystrophic. each of the latter three has several varieties.

  • Epidermolysis Bullosa Acquisita

    form of epidermolysis bullosa characterized by trauma-induced, subepidermal blistering with no family history of the disease. direct immunofluorescence shows immunoglobulin g deposited at the dermo-epidermal junction.

  • Epidermolysis Bullosa Dystrophica

    form of epidermolysis bullosa characterized by atrophy of blistered areas, severe scarring, and nail changes. it is most often present at birth or in early infancy and occurs in both autosomal dominant and recessive forms. all forms of dystrophic epidermolysis bullosa result from mutations in collagen type vii, a major component fibrils of basement membrane and epidermis.

  • Epidermolysis Bullosa Simplex

    a form of epidermolysis bullosa characterized by serous bullae that heal without scarring. mutations in the genes that encode keratin-5 and keratin-14 have been associated with several subtypes of epidermolysis bullosa simplex.

  • Epidermolysis Bullosa, Junctional

    form of epidermolysis bullosa having onset at birth or during the neonatal period and transmitted through autosomal recessive inheritance. it is characterized by generalized blister formation, extensive denudation, and separation and cleavage of the basal cell plasma membranes from the basement membrane.

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 Q81.1 to ICD-9-CMHistory

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

ICD-9-CM
757.39 Skin anomaly NEC
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 Q81.1Overview

Is Q81.1 (Epidermolysis bullosa) a billable code?

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

What MS-DRG does Q81.1 group to?

When epidermolysis bullosa letalis is the principal diagnosis on an inpatient stay, it groups to MS-DRG 595, 596, with relative weights from 1.0825 to 2.1207 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q81.1 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 epidermolysis bullosa letalis on inpatient claims.

What is the ICD-9 equivalent of Q81.1?

Under the General Equivalence Mappings, epidermolysis bullosa letalis converts to ICD-9-CM 757.39 (skin anomaly NEC). 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.