Version 2024

2024 ICD-10-CM Diagnosis Code L10

Pemphigus

ICD-10-CM Code:
L10
ICD-10 Code for:
Pemphigus
Is Billable?
Not Valid for Submission
Code Navigator:

Code Classification

  • Diseases of the skin and subcutaneous tissue
    (L00–L99)

L10 is a non-specific and non-billable diagnosis code code, consider using a code with a higher level of specificity for a diagnosis of pemphigus. The code is not specific and is NOT valid for the year 2024 for the submission of HIPAA-covered transactions. Category or Header define the heading of a category of codes that may be further subdivided by the use of 4th, 5th, 6th or 7th characters.

Specific Coding Applicable to Pemphigus

Non-specific codes like L10 require more digits to indicate the appropriate level of specificity. Consider using any of the following ICD-10-CM codes with a higher level of specificity when coding for pemphigus:

  • Use L10.0 for Pemphigus vulgaris - BILLABLE CODE

  • Use L10.1 for Pemphigus vegetans - BILLABLE CODE

  • Use L10.2 for Pemphigus foliaceous - BILLABLE CODE

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

  • Use L10.4 for Pemphigus erythematosus - BILLABLE CODE

  • Use L10.5 for Drug-induced pemphigus - BILLABLE CODE

  • L10.8 for Other pemphigus - NON-BILLABLE CODE

  • Use L10.81 for Paraneoplastic pemphigus - BILLABLE CODE

  • Use L10.89 for Other pemphigus - BILLABLE CODE

  • Use L10.9 for Pemphigus, unspecified - BILLABLE CODE

Clinical Information

  • 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.
  • 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.

Tabular List of Diseases and Injuries

The following annotation back-references are applicable to this diagnosis code. The Tabular List of Diseases and Injuries is a list of ICD-10-CM codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more.


Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.

Patient Education


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.

Pemphigoid is also an autoimmune skin disease. It leads to deep blisters that do not break easily. Pemphigoid is most common in older adults and may be fatal for older, sick patients.

Doctors diagnose pemphigus with a physical exam, a biopsy, and blood tests. The treatment of pemphigus and pemphigoid is the same: one or more medicines to control symptoms. These may include:

  • Steroids, which reduce inflammation
  • Drugs that suppress the immune system response
  • Antibiotics to treat associated infections

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases


[Learn More in MedlinePlus]

Pemphigus

Pemphigus is a rare disease that causes blistering on many parts of the body, including  the skin and the inside of the mouth, nose, throat, eyes, and genitals.
[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.