2026 ICD-10-CM Diagnosis Code L10.0Pemphigus vulgaris

ICD-10-CM Codes›L00–L99›L10-L14›L10

ICD-10-CM L10.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

L10.0 is a billable ICD-10-CM diagnosis code for pemphigus vulgaris. 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. Coders also document this condition as oral pemphigus vulgaris. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified inflammatory condition of skin.

Code Identity

ICD-10-CM Code
L10.0
Billable Status
Yes — Valid for Submission
Code Describes
Pemphigus vulgaris
Short Description
Pemphigus vulgaris
Same as the full description in the CMS dataset.
Parent Code
L10
Pemphigus

Code Classification

ChapterL00–L99Diseases of the skin and subcutaneous tissue
SectionL10-L14Bullous disorders
CategoryL10Pemphigus
This CodeL10.0Pemphigus vulgaris

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Oral pemphigus vulgaris
  • Pemphigus vulgaris
  • Pemphigus vulgaris of gingival mucous membrane

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.

    • Pemphigus
      • vulgaris

Clinical ClassificationClinical

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

CCSR SKN002
Other specified inflammatory condition of skin
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Desmoglein-3|130 kDa Pemphigus Vulgaris Antigen|Cadherin Family Member 6|DESMOGLEIN 3|DSG3|Desmoglein 3|PVA

    desmoglein-3 (999 aa, ~108 kda) is encoded by the human dsg3 gene. this protein plays a role in desmosome-mediated cell-cell adhesion.

  • DSG3 Antibody Positive|Anti-DSG3 Antibody Positive|Anti-Desmoglein 3 Antibody Positive|Anti-Desmoglein-3 Antibody Positive|Anti-PVA Antibody Positive|CDHF6 Antibody Positive|Cadherin Family Member 6 Antibody Positive|Desmoglein 3 Antibody Positive|Desmoglein-3 Antibody Positive|PVA Antibody Positive|Pemphigus Vulgaris Antigen Antibody Positive

    an indication that antibodies that recognize desmoglein-3 (dsg3) have been detected in a sample.

  • DSG3 wt Allele|ABOLM|CDHF6|Desmoglein 3 (Pemphigus Vulgaris Antigen) Gene|Desmoglein 3 wt Allele|PVA|Pemphigus Vulgaris Antigen Gene

    human dsg3 wild-type allele is located in the vicinity of 18q12.1 and is approximately 31 kb in length. this allele, which encodes desmoglein-3 protein, is involved in the modulation of cell-cell adhesion.

  • Mucosal-Dominant Pemphigus Vulgaris|Mucosal Dominant Pemphigus Vulgaris|mPV

    a subtype of pemphigus vulgaris in which lesions are predominantly found on mucosal membranes with relative sparing of cutaneous tissues.

  • Pemphigus Vulgaris

    an autoimmune blistering disorder. it is characterized by the presence of painful blisters and erosions in the skin and mucous membranes.

Patient EducationClinical

Pemphigus

Pemphigus is an autoimmune disorder. If you have it, your immune system attacks healthy cells in your skin and mouth, causing blisters and sores. No one knows the cause. Pemphigus does not spread from person to person. It does not appear to be inherited. But some people's genes put them more at risk for pemphigus.

Read the full article at MedlinePlus

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

Convert L10.0 to ICD-9-CMHistory

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

ICD-9-CM
694.4 Pemphigus
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 L10.0Overview

Is L10.0 (Pemphigus) a billable code?

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

What MS-DRG does L10.0 group to?

When pemphigus vulgaris 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.

What is the ICD-9 equivalent of L10.0?

Under the General Equivalence Mappings, pemphigus vulgaris converts to ICD-9-CM 694.4 (pemphigus). 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.