ICD-11 classes
14 Diseases of the skin
Malignant neoplasms involving the skin
Cutaneous markers of internal malignancy
EL10 — Paraneoplastic syndromes involving skin
EB40.2 — Paraneoplastic pemphigus

ICD-11 EB40.2 — Paraneoplastic pemphigus

Paraneoplastic pemphigus is a severe, often fatal autoimmune disease characterised by blisters and erosions not only on the skin and the mucous membranes but also involving other organs including the respiratory system. Auto-antibodies to a variety of plakin components of desmosomes and hemidesmosomes and to the protease inhibitor, Alpha-2-macroglobulin-like-1 protein, have all been implicated in its pathogenesis. It is strongly associated with lymphoproliferative disease.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Pemphigus #15786
• Paraneoplastic syndromes involving skin #17429