{"title":"PAPA样综合征与高须动脉炎对皮质类固醇的反应:1例报告","authors":"N. Dhattarwal","doi":"10.23880/cdoaj-16000301","DOIUrl":null,"url":null,"abstract":"Pyoderma gangrenosum, pyogenic sterile arthritis and acne (PAPA syndrome) is a hereditary, autosomal dominant, auto- inflammatory disease caused by missense mutation in the proline/serine/threonine phosphatase-interacting protein 1 (PSTPIP1) gene and predominantly affecting the joints and skin. Many reports now suggest that the clinical phenotype of PAPA syndrome may vary among people carrying the mutation and can lack the classic triad of pyogenic sterile arthritis, Pyoderma gangrenosum and cystic acne. No definite treatment strategy has been established for this PAPA like syndrome so far. We describe the treatment response of corticosteroids in a 23-year-old male with PAPA like syndrome and Takayasu arteritis, an association which has never been reported before.","PeriodicalId":164845,"journal":{"name":"Clinical Dermatology Open Access Journal","volume":"41 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"PAPA like Syndrome Associated with Takayasu Arteritis Responding to Corticosteroids: A Case Report\",\"authors\":\"N. Dhattarwal\",\"doi\":\"10.23880/cdoaj-16000301\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Pyoderma gangrenosum, pyogenic sterile arthritis and acne (PAPA syndrome) is a hereditary, autosomal dominant, auto- inflammatory disease caused by missense mutation in the proline/serine/threonine phosphatase-interacting protein 1 (PSTPIP1) gene and predominantly affecting the joints and skin. Many reports now suggest that the clinical phenotype of PAPA syndrome may vary among people carrying the mutation and can lack the classic triad of pyogenic sterile arthritis, Pyoderma gangrenosum and cystic acne. No definite treatment strategy has been established for this PAPA like syndrome so far. We describe the treatment response of corticosteroids in a 23-year-old male with PAPA like syndrome and Takayasu arteritis, an association which has never been reported before.\",\"PeriodicalId\":164845,\"journal\":{\"name\":\"Clinical Dermatology Open Access Journal\",\"volume\":\"41 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1900-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical Dermatology Open Access Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.23880/cdoaj-16000301\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Dermatology Open Access Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.23880/cdoaj-16000301","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
PAPA like Syndrome Associated with Takayasu Arteritis Responding to Corticosteroids: A Case Report
Pyoderma gangrenosum, pyogenic sterile arthritis and acne (PAPA syndrome) is a hereditary, autosomal dominant, auto- inflammatory disease caused by missense mutation in the proline/serine/threonine phosphatase-interacting protein 1 (PSTPIP1) gene and predominantly affecting the joints and skin. Many reports now suggest that the clinical phenotype of PAPA syndrome may vary among people carrying the mutation and can lack the classic triad of pyogenic sterile arthritis, Pyoderma gangrenosum and cystic acne. No definite treatment strategy has been established for this PAPA like syndrome so far. We describe the treatment response of corticosteroids in a 23-year-old male with PAPA like syndrome and Takayasu arteritis, an association which has never been reported before.