重度溃疡性结肠炎合并卡波西肉瘤1例并文献综述

卡波西肉瘤是与人类疱疹病毒-8感染相关的血管增生性疾病,多见于免疫功能低下的人群,服用免疫抑制剂或糖皮质激素可能是导致人类免疫缺陷病毒阴性患者发生卡波西肉瘤的原因之一.本文报告1例接受糖皮质激素治疗的重度溃疡性结肠炎患者发生的皮肤卡波西肉瘤,并进行文献综述.患者男性,64岁,诊断溃疡性结肠炎1年,因重度溃疡性结肠炎发作接受激素治疗,4个多月后患者躯干,四肢出现多发暗紫色丘疹,结节,质地坚硬,下肢为著,经皮肤组织活检病理学检查提示皮肤卡波西肉瘤,免疫组织化学检测显示人类疱疹病毒-8染色阳性,经停用糖皮质激素,并行化疗后病情缓解.分别在万方数据知识服务平台和中国知网以"卡波西肉瘤"和"炎症性肠病"为检索词检索相关文献,未检索到中文文献.在PubMed上以("ulcerative colitis" OR "Crohn's disease" OR "inflammatory bowel disease") AND (Kaposi sarcoma)为检索词检索,共检索到38篇英文文献,另从相关文献中补充,共检索到25例与炎症性肠病相关的卡波西肉瘤,连同本例的26例患者中,男性占绝大多数(80.8%,21/26).平均年龄(51.1±16.4)岁,溃疡性结肠炎20例,克罗恩病6例.22例患者报告了人类免疫缺陷病毒检测结果,均为阴性.所有患者均使用过至少一种免疫功能调节剂,包括糖皮质激素,硫唑嘌呤/6-巯基嘌呤,氨甲喋呤,环孢菌素,抗肿瘤坏死因子α单克隆抗体.14例报告人疱疹病毒-8结果的患者中,13例阳性,1例阴性.病变单纯累及肠道者18例,单纯累及皮肤者3例,5例患者同时累及皮肤和肠道.25例报告了治疗方案,其中3例患者仅停用免疫调节剂,1例患者停药后接受放疗,1例患者停药后接受化疗,20例患者接受手术治疗,总体而言预后良好.炎症性肠病相关的卡波西肉瘤往往与使用激素,免疫抑制剂和生物制剂有关.鉴别卡波西肉瘤,炎症性肠病相关和药物相关的皮肤表现至关重要.此外,在诊疗过程中重视多学科团队的协同作用,能够更早,更准确地对少见病例做出诊断和治疗.

Kaposi sarcoma combined with severe ulcerative colitis: A case report and literature review

SUMMARY　Kaposi’s sarcoma (KS) is an unusual vascular tumor associated with human herpesvirus-8 (HHV-8) infection, which is common in immunosuppressors. Although extremely rare, iatrogenic (drug-related) KS can occur in human immunodeficiency virus (HIV)-negative patients under immunosuppressive therapy. We report a 64-year-old male diagnosed with ulcerative colitis for 1 year. He was treated with methylprednisolone because of an acute severe disease flare. He presented with several popular violet lesions on the body 4 months after steroid therapy. Histological examination of skin biopsies showed Kaposi’s sarcoma associated with HHV-8. The skin lesions regressed after steroid withdrawal and chemotherapy. Two key words “Kaposi’s sarcoma” and “inflammatory bowel disease” were searched in Wanfang data and CNKI, but no relevant articles were found. Thirty-eight articles in English were retrieved on PubMed with the key words of (“ulcerative colitis” OR “Crohn’s disease” OR “inflammatory bowel disease”) AND (Kaposi sarcoma). Twenty-five cases of Kaposi’s sarcoma related to inflammatory bowel disease (IBD) were reported. Including this case, the majority of 26 Kaposi’s sarcoma related IBD patients were male (80.8%, 21/26). The average age was (51.1 ± 16.4) years. Twenty cases were ulcerative colitis and 6 were Crohn’s disease. All the patients received immunomodulatory therapy, including glucocorticoid, azathioprine/mercaptopurine, methotrexate, cyclosporin and anti tumor necrosis factor α antibody. Thirteen cases were positive for HHV-8. There were 18 cases involving the distal ileum and colorectum only, 3 cases involving skin only, and 5 cases involving both skin and colorectum at the same time. Overall, the prognosis was good. Three patients only stopped immunosuppressive therapy, 1 received radiotherapy, 1 received chemotherapy, and 20 received surgery. Kaposi’s sarcoma could be seen in IBD patients with immunomodulatory therapy. It is very important to distinguish from the skin lesions related to IBD or drug treatment. The adverse reactions of immunomodulatory therapy should not be ignored. In addition, attention should be paid to the cooperation of multi-disciplinary team, which can diagnose and treat rare cases earlier and more accurately.