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  2. ICD-10-CM Codes List
  3. L00–L99
  4. L10-L14
  5. Pemphigus (L10)

Pemphigus (L10)

ICD-10 codes beginning with L10 cover the diagnosis of pemphigus, a rare group of autoimmune skin disorders characterized by blistering. These codes help specify the exact type of pemphigus present for accurate medical documentation and treatment planning.

These codes range from the general L10 for pemphigus to detailed subtypes like L10.0 for pemphigus vulgaris, commonly referred to as "oral pemphigus vulgaris" or "pemphigus vulgaris of gingival mucous membrane." Other variations include L10.1 for pemphigus vegetans, also called "Neumann type," and L10.2 for pemphigus foliaceous (sometimes spelled "pemphigus foliaceus"). The classification includes less common forms such as L10.3 Brazilian pemphigus, known as "fogo selvagem," and L10.4 pemphigus erythematosus. Drug-related cases are coded under L10.5. For instances related to cancer, L10.81 represents paraneoplastic pemphigus. Finally, codes L10.8, L10.89, and L10.9 cover other specified and unspecified pemphigus types, helping coders pinpoint diagnoses like IgA pemphigus or prebullous pemphigus. Using these ICD-10 codes for pemphigus ensures clear identification of the exact subtype, facilitating targeted treatment and appropriate medical billing.

Instructional Notations

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.

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

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.