2027 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 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q81.0 is a billable ICD-10-CM diagnosis code for epidermolysis bullosa simplex. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) 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
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 the other POA exempt codes in Other congenital malformations (Q80-Q89).

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

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q81.0: its own notes plus those printed at Chapter 17. 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.

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.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name Q81.0, its category, or a range that includes it.

Excludes1 2

These codes carry an Excludes1 note naming Q81.0: they are not reported together with it, unless the two conditions are unrelated.

  • L12.3 Acquired epidermolysis bullosa
    epidermolysis bullosa (congenital) (Q81.-) names Q81.-, which includes this code
  • P83 Other conditions of integument specific to newborn
    congenital malformations of skin and integument (Q80-Q84) names Q80-Q84, which includes this code

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.

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.

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 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q81.0Overview

What is the ICD-10 code for epidermolysis bullosa simplex?

The ICD-10-CM code for epidermolysis bullosa simplex is Q81.0 (sometimes written as Q810). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

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, 2026 through September 30, 2027.

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 596, 595, with relative weights from 1.1067 to 2.0230 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. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.

Can Q81.0 be reported with Q87.19?

Not as a rule. The Excludes1 note on Q81.0 lists "Cockayne's syndrome (Q87.19)". 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).