EB40

ICD-11 MMS 2026-01

Pemphigus

Description

Pemphigus is a group of chronic autoimmune skin diseases characterised by blister formation on the skin and the mucous membranes. The exact causes of the disease are unknown but the disease is mediated by auto-antibodies to desmosome components. Three clinical forms have been characterised. Pemphigus vulgaris, pemphigus foliaceus and pemphigus vegetans. Other variants exist, namely intercellular IgA dermatosis and paraneoplastic pemphigus.

Exclusions from above levels

  • 1B72.0 – Bullous impetigo of the napkin area
  • 1F23.12 – Napkin candidosis
  • EH71 – Dermatoses precipitated by drug therapy
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  • EH76 – Dermatoses associated with specific classes of medication

Child codes

  • EB40.0 – Pemphigus vulgaris
  • EB40.1 – Pemphigus foliaceus
  • EB40.2 – Paraneoplastic pemphigus
  • EB40.Y – Other specified pemphigus
  • EB40.Z – Pemphigus, unspecified

Foundation children elsewhere

Sibling codes

  • EB41 – Pemphigoid
  • EB42 – Linear IgA bullous dermatosis
  • EB43 – Epidermolysis bullosa acquisita
  • EB44 – Dermatitis herpetiformis
  • EB4Y – Other specified immunobullous disorder

Foundation entity

Pemphigus – http://id.who.int/icd/entity/191659986

Part of: 14: Diseases of the skin