Abstract:
Yao syndrome (YAOS) is a novel systemic autoinflammatory disease linked to the nucleotide-binding oligomerization domain (NOD2) gene. It is characterized by periodic fevers, gastrointestinal (GI) symptoms, arthritis, and dermatitis, among other symptoms. A sparse literature exists on this disease, and little is known about its dermatological manifestations. A review of available literature was performed to characterize the cutaneous manifestations of Yao syndrome. Cutaneous manifestations were documented in 85.7% of patients, with common characteristic descriptions of erythematous patches and plaques involving the face, trunk, abdomen, and extremities. Based on our review of treatment modalities employed for Yao syndrome, prednisone is an appropriate initial approach, with oral sulfasalazine and other disease-modifying antirheumatic drugs serving as appropriate secondary options. YAOS should be considered in the differential diagnosis of patients presenting with a dermatitic rash, especially in the context of concurrent articular symptoms, periodic fever, and GI symptoms.
摘要:
Yao综合征(YAOS)是一种与核苷酸结合寡聚化结构域(NOD2)基因相关的新型全身性自身炎性疾病。它的特点是周期性发烧,胃肠道(GI)症状,关节炎,和皮炎,在其他症状中。关于这种疾病的文献很少,对其皮肤病学表现知之甚少。对现有文献进行了综述,以描述Yao综合征的皮肤表现。85.7%的患者有皮肤表现,具有涉及面部的红斑和斑块的共同特征性描述,树干,腹部,和四肢。根据我们对姚氏综合征的治疗方式的回顾,泼尼松是一种适当的初始方法,口服柳氮磺吡啶和其他缓解疾病的抗风湿药物作为适当的次要选择。YAOS应考虑在鉴别诊断患者出现皮肤皮疹,特别是在并发关节症状的情况下,周期性发烧,和胃肠道症状。