期刊
INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE
卷 47, 期 6, 页码 -出版社
SPANDIDOS PUBL LTD
DOI: 10.3892/ijmm.2021.4951
关键词
generalized pustular psoriasis; mutation; IL36RN gene; CARD14 gene; MPO gene; pathoimmunology; biologics treatment; heterogeneity
资金
- Sichuan Science and Technology Program [2019YFS0332, 2019YFS0038, 2020YFQ0045]
Generalized pustular psoriasis is a rare and severe inflammatory skin disease characterized by recurrent pustular eruptions. Recent studies have identified genetic variants associated with the disease, and biological agents targeting the IL-36 pathway show promise as potential treatments.
Generalized pustular psoriasis (GPP) is a rare and severe auto-inflammatory skin disease that is characterized by recurrent, acute onset, and generalized pustular eruptions on erythematous, inflamed skin. GPP is traditionally classified as a variant of psoriasis vulgaris, even though recent clinical, histological and genetic evidence suggests that it is a heterogeneous disease and requires a separate diagnosis. In recent years, variants of IL36RN, CARD14, AP1S3 and MPO genes have been identified as causative or contributing to genetic defects in a proportion of patients affected by GPP. These disease-related genes are involved in common inflammatory pathways, in particular in the IL-1/IL-36-chemokines-neutrophil pathogenic axis. At present, no standard therapeutic guidelines have been established for GPP management, and there is a profound need for novel efficacious treatments of GPP. Among them, biological agents antagonizing the IL-36 pathway are promising therapeutics. The aim of the present review is to provide the most recent updates on the genetics, genotype-phenotype correlation and pathological basis of GPP, as well as on biologic treatments available for GPP and relative clinical courses.
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