ICD-11 classes
14 Diseases of the skin
Inflammatory dermatoses
Immunobullous diseases of the skin
EB40 — Pemphigus

ICD-11 EB40 — Pemphigus

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code EB40 contains 6 clarifying diagnoses:

1. EB40.0 — Pemphigus vulgaris
   It contains 3 clarifying diagnoses.
2. EB40.1 — Pemphigus foliaceus
3. EB40.2 — Paraneoplastic pemphigus
4. KA07.1 — Neonatal pemphigus
5. EB40.Y — Other specified pemphigus
6. EB40.Z — Pemphigus, unspecified