Dhanya Dhanesh, Sabeena Jayapalan, P. Nair, Athira Sudhesan
{"title":"坏疽脓皮病合并马凡氏综合征和克罗恩病:一种罕见的关联","authors":"Dhanya Dhanesh, Sabeena Jayapalan, P. Nair, Athira Sudhesan","doi":"10.25259/jsstd_20_2023","DOIUrl":null,"url":null,"abstract":"A 22-year-old man presented with multiple, painful ulcers on the thighs, legs, and face of 10-day duration and diarrhea and bleeding per rectum of 2-year duration. The ulcers were tender and had a peripheral violaceous border. General examination revealed tall stature, long extremities, kyphosis, arachnodactyly, and hyperextensible wrist and thumbs. The arm span was more than the height and the thumb sign and wrist sign were positive, diagnostic of Marfan syndrome. Skin biopsy from the edge of the ulcer showed infiltrates of neutrophils and lymphocytes in the dermis and subcutaneous tissue. A detailed evaluation ruled out other causes for skin ulceration and we made a diagnosis of pyoderma gangrenosum. Echocardiogram demonstrated mitral valve prolapse. Computed tomography of abdomen showed segmental mucosal thickening as skip lesions in the colon and rectum. Endoscopy showed chronic colitis with multiple punched out ulcers. Colonic biopsy showed noncaseating granulomas suggestive of Crohn’s disease. Although inflammatory bowel disease is a well-known association of pyoderma gangrenosum, we did not come across any previous reports of coexistence of pyoderma gangrenosum, Crohn’s disease, and Marfan syndrome.","PeriodicalId":17051,"journal":{"name":"Journal of Skin and Sexually Transmitted Diseases","volume":"89 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pyoderma gangrenosum with Marfan syndrome and Crohn’s disease: A rare association\",\"authors\":\"Dhanya Dhanesh, Sabeena Jayapalan, P. Nair, Athira Sudhesan\",\"doi\":\"10.25259/jsstd_20_2023\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"A 22-year-old man presented with multiple, painful ulcers on the thighs, legs, and face of 10-day duration and diarrhea and bleeding per rectum of 2-year duration. The ulcers were tender and had a peripheral violaceous border. General examination revealed tall stature, long extremities, kyphosis, arachnodactyly, and hyperextensible wrist and thumbs. The arm span was more than the height and the thumb sign and wrist sign were positive, diagnostic of Marfan syndrome. Skin biopsy from the edge of the ulcer showed infiltrates of neutrophils and lymphocytes in the dermis and subcutaneous tissue. A detailed evaluation ruled out other causes for skin ulceration and we made a diagnosis of pyoderma gangrenosum. Echocardiogram demonstrated mitral valve prolapse. Computed tomography of abdomen showed segmental mucosal thickening as skip lesions in the colon and rectum. Endoscopy showed chronic colitis with multiple punched out ulcers. Colonic biopsy showed noncaseating granulomas suggestive of Crohn’s disease. Although inflammatory bowel disease is a well-known association of pyoderma gangrenosum, we did not come across any previous reports of coexistence of pyoderma gangrenosum, Crohn’s disease, and Marfan syndrome.\",\"PeriodicalId\":17051,\"journal\":{\"name\":\"Journal of Skin and Sexually Transmitted Diseases\",\"volume\":\"89 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-08-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Skin and Sexually Transmitted Diseases\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.25259/jsstd_20_2023\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Skin and Sexually Transmitted Diseases","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.25259/jsstd_20_2023","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Pyoderma gangrenosum with Marfan syndrome and Crohn’s disease: A rare association
A 22-year-old man presented with multiple, painful ulcers on the thighs, legs, and face of 10-day duration and diarrhea and bleeding per rectum of 2-year duration. The ulcers were tender and had a peripheral violaceous border. General examination revealed tall stature, long extremities, kyphosis, arachnodactyly, and hyperextensible wrist and thumbs. The arm span was more than the height and the thumb sign and wrist sign were positive, diagnostic of Marfan syndrome. Skin biopsy from the edge of the ulcer showed infiltrates of neutrophils and lymphocytes in the dermis and subcutaneous tissue. A detailed evaluation ruled out other causes for skin ulceration and we made a diagnosis of pyoderma gangrenosum. Echocardiogram demonstrated mitral valve prolapse. Computed tomography of abdomen showed segmental mucosal thickening as skip lesions in the colon and rectum. Endoscopy showed chronic colitis with multiple punched out ulcers. Colonic biopsy showed noncaseating granulomas suggestive of Crohn’s disease. Although inflammatory bowel disease is a well-known association of pyoderma gangrenosum, we did not come across any previous reports of coexistence of pyoderma gangrenosum, Crohn’s disease, and Marfan syndrome.