2026 ICD-10-CM Diagnosis Code L10Pemphigus

ICD-10-CM Codes›L00–L99›L10-L14›L10

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

L10 is a non-billable ICD-10-CM category code for pemphigus, so it cannot be submitted on claims. Use a more specific code from this category instead, such as L10.0, L10.1, L10.2, and L10.3.

Code Identity

ICD-10-CM Code
L10
Billable Status
No — Non-Billable Category
Code Describes
Pemphigus
Chapter
L10-L14
Bullous disorders

Code Classification

ChapterL00–L99Diseases of the skin and subcutaneous tissue
SectionL10-L14Bullous disorders
CategoryL10Pemphigus
This CodeL10Pemphigus

Specific Coding for PemphigusOverview

Non-specific codes like L10 require more characters. Use one of these billable codes instead:

  • Use L10.0 for Pemphigus vulgaris

  • Use L10.1 for Pemphigus vegetans

  • Use L10.2 for Pemphigus foliaceous

  • Use L10.3 for Brazilian pemphigus [fogo selvagem]

  • Use L10.4 for Pemphigus erythematosus

  • Use L10.5 for Drug-induced pemphigus

  • L10.8 for Other pemphigus

  • Use L10.81 for Paraneoplastic pemphigus

  • Use L10.89 for Other pemphigus

  • Use L10.9 for Pemphigus, unspecified

Tabular List NotesGuidance

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

Type 1 Excludes

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.

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 L10Overview

Is L10 (Pemphigus) a billable code?

No. This is a category header that groups the codes for pemphigus, and headers cannot be submitted on claims. Claims for pemphigus need a more specific code from this category, such as L10.0, L10.1, and L10.2.