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.;
Origin ID : 104197957;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
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.
