Paraneoplastic pemphigus (Q43773): Difference between revisions
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Pênfigo paraneoplásico é uma doença autoimune grave, frequentemente fatal, caracterizada por bolhas e erosões não apenas na pele e nas mucosas, mas também envolvendo outros órgãos, incluindo o sistema respiratório. Autoanticorpos para uma variedade de componentes de plaquinas de desmossomos e hemidesmossomos e para o inibidor de protease, proteína alfa-2-macroglobulina-like-1, foram todos implicados em sua patogênese. Está fortemente associada a doença linfoproliferativa. | |||
| description / en | description / en | ||
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. | |||
Revision as of 10:54, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | EB40.2 |
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| English | 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. |
