Bowel-Associated Dermatosis-Arthritis Syndrome in an Adolescent With Short Bowel Syndrome
Bowel-Associated Dermatosis-Arthritis Syndrome in an Adolescent With Short Bowel Syndrome
复制标题
DOI：
10.1097/rhu.0000000000000137
复制
发表时间：
2014-09-01
影响因子：
3.4
通讯作者：
Salgado, Manuel
中科院分区：
文献类型：
--
作者：
Pereira, Ester;Estanqueiro, Paula;Salgado, Manuel
肠相关性皮肤病-关节炎综合征（BADAS）是一种嗜中性皮肤病，其特征是发生关节炎和与肠道疾病相关的皮肤病变，无论是否有肠旁路。我们报告一个不寻常的情况下，BADAS在一个15岁的白色男性先天性无神经节细胞症的结肠和hypoganglionosis的小肠和多次肠道手术在儿童并发短肠综合征。他表现为复发性外周多发性关节炎、腱鞘炎和位于腿背外侧和足背的疼痛性肿胀性皮下结节。组织学检查发现一种嗜酸性皮肤病，证实了BADAS的诊断。尽管BADAS是一种罕见的疾病，尤其是在儿科年龄，但在患有肠病的儿童和青少年中引起BADAS的诊断是很重要的，因为治疗选择和预后与其他风湿性疾病不同。
Bowel-associated dermatosis-arthritis syndrome (BADAS) is a neutrophilic dermatosis, characterized by the occurrence of arthritis and skin lesions related to bowel disease with or without bowel bypass. We report an unusual case of BADAS in a 15-year-old white male with congenital aganglionosis of the colon and hypoganglionosis of the small intestine and multiple bowel surgeries in childhood complicated by short bowel syndrome. He presented with recurrent peripheral polyarthritis, tenosynovitis, and painful erythematous subcutaneous nodules located on the dorsolateral regions of the legs and on the dorsa of the feet. Histological examination disclosed a neutrophilic dermatosis confirming the diagnosis of BADAS.Although an uncommon disease, especially at pediatric age, it is important to evoke the diagnosis of BADAS in children and adolescents with bowel disease, because treatment options and prognosis are distinct from other rheumatologic conditions.