loading...| Preferred Name |
副肿瘤性天疱疮 / Paraneoplastic pemphigus |
| Definitions |
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. |
| ID |
http://purl.bmicc.cn/ontology/ICD11CN/EB40.2 |
| 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. |
| hasDbXref | |
| label |
副肿瘤性天疱疮 / Paraneoplastic pemphigus |
| mappingRelation | |
| notation |
EB40.2 |
| prefixIRI |
ICD11CN:EB40.2 |
| prefLabel |
副肿瘤性天疱疮 / Paraneoplastic pemphigus |
| subClassOf |