EB40.2 Paraneoplastic pemphigus

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2025-01

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.

postcoordination

synonyms

• Paraneoplastic pemphigus

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