A Síndrome de CANDLE

Palavras-chave: Complexo de Endopeptidases do Proteassoma/genética, Doenças Hereditárias Autoinflamatórias, Doenças da Pele/genética, Lipodistrofia, Síndrome

Resumo

A síndrome de CANDLE, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature, é uma doença auto-inflamatória crónica, fisiopatologicamente condicionada pela disfunção intracelular do proteasoma/imunoproteasoma. Esta revisão descreve o reconhecimento da síndrome de CANDLE, os desenvolvimentos na compreensão do seu mecanismo patológico, os antecedentes genéticos e as estratégias terapêuticas emergentes para esta condição.

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Referências

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Publicado
2020-05-06
Como Citar
Kieselová, K., Santiago, F., & Henrique, M. (2020). A Síndrome de CANDLE. Revista Da Sociedade Portuguesa De Dermatologia E Venereologia, 78(1), 17-24. https://doi.org/10.29021/spdv.78.1.1132
Secção
Educação Médica Contínua