2026 ICD-10-CM Diagnosis Code Q81.0Epidermolysis bullosa simplex

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q81

ICD-10-CM Q81.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q81.0 is a billable ICD-10-CM diagnosis code for epidermolysis bullosa simplex. 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. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q81.0
Billable Status
Yes — Valid for Submission
Code Describes
Epidermolysis bullosa simplex
Short Description
Epidermolysis bullosa simplex
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 CodeQ81.0Epidermolysis bullosa simplex

Present on Admission (POA)Billing

Q81.0 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.

  • Autosomal dominant epidermolysis bullosa simplex
  • Autosomal recessive epidermolysis bullosa simplex
  • Basal epidermolysis bullosa simplex
  • Epidermolysis bullosa simplex
  • Epidermolysis bullosa simplex due to BP230 deficiency
  • Epidermolysis bullosa simplex due to exophilin 5 deficiency
  • Epidermolysis bullosa simplex due to plakophilin deficiency
  • Epidermolysis bullosa simplex herpetiformis
  • Epidermolysis bullosa simplex with circinate migratory erythema
  • Epidermolysis bullosa simplex with hypodontia
  • Epidermolysis bullosa simplex with mottled pigmentation
  • Epidermolysis bullosa simplex with muscular dystrophy
  • Epidermolysis bullosa simplex with pyloric atresia
  • Epidermolysis bullosa simplex, Ogna type
  • Epidermolysis simplex superficialis
  • Gastric atresia
  • Generalized epidermolysis bullosa simplex
  • Intermediate epidermolysis bullosa simplex with cardiomyopathy
  • Keratoderma due to Dowling-Meara type epidermolysis bullosa simplex
  • KRT14 related epidermolysis bullosa simplex
  • Lethal acantholytic erosive disorder
  • Lethal autosomal recessive epidermolysis bullosa simplex
  • Pyloric atresia
  • Suprabasal epidermolysis bullosa simplex

Tabular List NotesGuidance

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

Type 1 Excludes

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)
        • simplex

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 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 Simplex

    a genetic skin disorder caused by mutations in the krt5 and krt14 genes. it is characterized by the formation of blisters and increased fragility of the skin.

  • Intermediate Epidermolysis Bullosa Simplex with Cardiomyopathy

    an autosomal dominant condition caused by mutation(s) in the klhl24 gene, encoding kelch-like protein 24. it is characterized by epidermolysis bullosa and dilated cardiomyopathy.

  • KRT5 wt Allele|CK5|DDD|DDD1|EBS2|Epidermolysis Bullosa Simplex 2 Dowling-Meara/Kobner/Weber-Cockayne Types Gene|K5|KRT5A|Keratin 5 (Epidermolysis Bullosa Simplex, Dowling-Meara/Kobner/Weber-Cockayne Types) Gene|Keratin 5 wt Allele|Keratin 5, Type II Gene

    human krt5 wild-type allele is located in the vicinity of 12q13.13 and is approximately 6 kb in length. this allele, which encodes keratin, type ii cytoskeletal 5 protein, plays a role in the formation of the epidermis. mutation of the gene is associated with dowling-degos disease and several subtypes of epidermolysis bullosa simplex.

  • Plectin|Epidermolysis Bullosa Simplex 1 (Ogna)|Hemidesmosomal Protein 1|Plectin 1, Intermediate Filament Binding Protein 500kDa|Plectin-1

    plectin (4684 aa, ~53 kda) is encoded by the human plec gene. this protein plays a role in linking intermediate filaments to desmosomes or hemidesmosomes.

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 Q81.0 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 Q81.0Overview

Is Q81.0 (Epidermolysis bullosa) a billable code?

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

What MS-DRG does Q81.0 group to?

When epidermolysis bullosa simplex 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 Q81.0 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 simplex on inpatient claims.

What is the ICD-9 equivalent of Q81.0?

Under the General Equivalence Mappings, epidermolysis bullosa simplex 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.