Fonseca Ashley, Sunny Julee, Felipez Lina M
Department of Medical Education, Nicklaus Children's Hospital, Miami, FL, USA.
Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Nicklaus Children's Hospital, Miami, FL, USA.
Case Rep Pediatr. 2021 May 20;2021:1996430. doi: 10.1155/2021/1996430. eCollection 2021.
Inflammatory bowel disease (IBD) that presents in children <6 years of age is known as very early-onset IBD (VEO-IBD). Extraintestinal manifestations in IBD, such as erythema nodosum (EN), pyoderma gangrenosum (PG), and, less likely, leukocytoclastic vasculitis (LV), are more commonly present in Crohn's disease. Association between LV and ulcerative colitis (UC) is not commonly seen. We report a case of a 6-year-old female with a VEO-IBD UC phenotype presenting with multiple episodes of leukocytoclastic vasculitis, each preceded by streptococcal pharyngitis. Prior to the diagnosis of VEO-IBD, a skin biopsy was obtained and had shown leukocytoclastic vasculitis with a negative IgA stain. Initial laboratory results were remarkable for leukocytosis and increased anti-strep O and anti-DNase B titers. Gastrointestinal panel PCR demonstrated toxin A/B. Treatment for LV consisted of methylprednisolone IV 20 mg for four days with a weaning schedule of prednisolone for two weeks and naproxen 250 mg BID for three days. was treated with metronidazole 250 mg TID for ten days. She remained stable for three years until she presented with continuous bloody stools, newly onset chest pain, and shortness of breath. Computed tomography angiogram (CTA) was normal. Stool calprotectin was elevated at 658 mcg/gm. Abdominal magnetic resonance enterography (MRE), esophagogastroduodenoscopy, and colonoscopy confirmed a VEO-IBD ulcerative colitis phenotype. She was started on infliximab 10 mg/kg every four weeks after infliximab titers, and antibodies were obtained. Currently, the patient remains on clinical and biochemical remission, with no recent LV episodes or recurrence of streptococcal pharyngitis. Our patient is unique as no case report has been published with multiple episodes of leukocytoclastic vasculitis in association with a VEO-IBD UC phenotype.
6岁以下儿童出现的炎症性肠病（IBD）被称为极早发型IBD（VEO-IBD）。IBD的肠外表现，如结节性红斑（EN）、坏疽性脓皮病（PG），以及较少见的白细胞破碎性血管炎（LV），在克罗恩病中更常见。LV与溃疡性结肠炎（UC）之间的关联并不常见。我们报告一例6岁女性VEO-IBD UC表型患者，出现多次白细胞破碎性血管炎发作，每次发作前均有链球菌性咽炎。在诊断VEO-IBD之前，进行了皮肤活检，结果显示白细胞破碎性血管炎，IgA染色阴性。初始实验室检查结果显示白细胞增多，抗链球菌溶血素O和抗脱氧核糖核酸酶B滴度升高。胃肠道组PCR检测显示毒素A/B。LV的治疗包括静脉注射甲泼尼龙20mg，共4天，随后泼尼松龙逐渐减量2周，萘普生250mg，每日2次，共3天。用甲硝唑250mg，每日3次，共10天进行治疗。她保持稳定3年，直到出现持续便血、新发胸痛和呼吸急促。计算机断层血管造影（CTA）正常。粪便钙卫蛋白升高至658μg/g。腹部磁共振肠造影（MRE）、食管胃十二指肠镜检查和结肠镜检查证实为VEO-IBD溃疡性结肠炎表型。在检测英夫利昔单抗滴度并检测抗体后，开始每四周给予英夫利昔单抗10mg/kg治疗。目前，患者处于临床和生化缓解状态，近期无LV发作或链球菌性咽炎复发。我们的患者很独特，因为尚未有关于VEO-IBD UC表型合并多次白细胞破碎性血管炎发作的病例报告发表。
