2026 ICD-10-CM Diagnosis Code L10.4Pemphigus erythematosus

ICD-10-CM Codes›L00–L99›L10-L14›L10

ICD-10-CM L10.4
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

L10.4 is a billable ICD-10-CM diagnosis code for pemphigus erythematosus. 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. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified inflammatory condition of skin.

Code Identity

ICD-10-CM Code
L10.4
Billable Status
Yes — Valid for Submission
Code Describes
Pemphigus erythematosus
Short Description
Pemphigus erythematosus
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 CodeL10.4Pemphigus erythematosus

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Pemphigus erythematosus
  • Pemphigus foliaceus

Tabular List NotesGuidance

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

Inclusion Terms

  • Senear-Usher 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.

    • Pemphigus
      • erythematosus
    • Senear-Usher disease or syndrome
    • Usher-Senear disease or syndrome

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 1

    a desmosomal cadherin that is an autoantigen in the acquired skin disorder pemphigus foliaceus.

  • Pemphigus

    group of chronic blistering diseases characterized histologically by acantholysis and blister formation within the epidermis.

  • Pemphigus, Benign Familial

    an autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and bullae mainly on the neck, axillae, and groin. mutations in the atp2c1 gene (encoding the secretory pathway ca2++/mn2++ atpase 1 (spca1)) cause this disease. it is clinically and histologically similar to darier disease - both have abnormal, unstable desmosomes between keratinocytes and defective calcium-transporting atpases. it is unrelated to pemphigus vulgaris though it closely resembles that disease.

  • ATP2C1 wt Allele|ATP2C1A|ATPase Secretory Pathway Ca2+ Transporting 1 wt Allele|ATPase, Ca(2+)-Sequestering Gene|ATPase, Ca(2+)-Transporting, Type 2c, Member 1 Gene|ATPase, Ca++ Transporting, Type 2C, Member 1 Gene|BCPM|Benign Chronic Pemphigus (Hailey-Hailey Disease) Gene|HHD|HUSSY-28|KIAA1347|PMR1|PMR1, Rat, Homolog of Gene|SPCA1|Secretory Pathway Ca(2+) ATPase 1 Gene|Secretory Pathway Ca2+/Mn2+ ATPase 1 Gene|hSPCA1

    human atp2c1 wild-type allele is located in the vicinity of 3q22.1 and is approximately 166 kb in length. this allele, which encodes calcium-transporting atpase type 2c member 1 protein, plays a role in the transport of ions into the golgi to regulate the trafficking of newly synthesized proteins via the secretory pathway. mutation of the gene are associated with familial benign pemphigus.

  • Desmoglein-1|Cadherin Family Member 4|DESMOGLEIN 1|DG1|DGI|DSG1|Desmoglein 1|Desmosomal Glycoprotein 1|Pemphigus Foliaceus Antigen

    desmoglein-1 (1049 aa, ~114 kda) is encoded by the human dsg1 gene. this protein is involved in cell-cell adhesion and keratinization.

  • 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.

  • Desmoplakin|250/210 kDa Paraneoplastic Pemphigus Antigen|DP

    desmoplakin (2871 aa, ~332 kda) is encoded by the human dsp gene. this protein is involved in the maintenance of desmosome structure.

  • 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.

  • Familial Benign Pemphigus|Benign Familial Pemphigus|Hailey-Hailey Disease

    an autosomal dominant disorder characterized by a history of multiple relapses and remissions of pemphigus lesions.

  • 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

    a blistering skin disorder. morphologically it is characterized by acantholysis and intraepidermal blister formation.

  • 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 L10.4 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 L10.4Overview

Is L10.4 (Pemphigus) a billable code?

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

What MS-DRG does L10.4 group to?

When pemphigus erythematosus 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 L10.4?

Under the General Equivalence Mappings, pemphigus erythematosus 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.