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

Dhanya Dhanesh, Sabeena Jayapalan, P. Nair, Athira Sudhesan
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引用次数: 0

摘要

22岁男性,大腿、腿部和面部出现多处疼痛溃疡,持续10天,直肠腹泻和出血持续2年。溃疡触痛,周围呈紫色边界。一般检查显示身高,四肢长,后凸,蛛网膜畸形,手腕和拇指超伸直。臂展大于身高,拇指征和腕征阳性,诊断马凡氏综合征。溃疡边缘的皮肤活检显示真皮和皮下组织中有中性粒细胞和淋巴细胞浸润。详细的评估排除了皮肤溃疡的其他原因,我们诊断为坏疽性脓皮病。超声心动图显示二尖瓣脱垂。腹部计算机断层扫描显示结肠和直肠的节段性粘膜增厚。内窥镜检查显示慢性结肠炎伴多处穿孔溃疡。结肠活检显示非干酪化肉芽肿提示克罗恩病。虽然炎症性肠病是众所周知的坏疽性脓皮病的关联,但我们没有遇到任何先前关于坏疽性脓皮病、克罗恩病和马凡氏综合征共存的报道。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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.
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