" /> Paraneoplastic pemphigus - CISMeF





ICD-11 code : EB40.2;

Preferred Label : Paraneoplastic pemphigus;

ICD-11 definition : Paraneoplastic pemphigus is a severe, often fatal autoimmune disease characterized 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.;

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Paraneoplastic pemphigus is a severe, often fatal autoimmune disease characterized 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.

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28/07/2025


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