Acquired idiopathic generalized anhidrosis (AIGA) is a rare disorder characterized by insidious or sudden onset of the inability to sweat involving >25% of body surface area in the absence of other neurologic or sweat gland abnormalities and typically affects young, healthy, Asian men. Here, we describe two Caucasian teenagers with the diagnosis. They both had variable responses to prednisone, one in the setting of an elevated ANA, suggesting an autoimmune or inflammatory pathomechanism of the disorder. It is essential the clinician recognizes this rare entity and initiates timely intervention to prevent the serious consequences of hyperpyrexia.

中文翻译：

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获得性特发性全身性无汗症：两名白人患者的病例系列

获得性特发性全身性无汗症 (AIGA) 是一种罕见的疾病，其特征是在没有其他神经系统或汗腺异常的情况下，隐匿或突然出现不能出汗的情况，涉及超过 25% 的体表面积，通常影响年轻、健康的亚洲男性。在这里，我们描述了两名白人青少年的诊断。他们都对泼尼松有不同的反应，一种是在 ANA 升高的情况下，表明该疾病的自身免疫或炎症病理机制。临床医生必须认识到这种罕见疾病并及时采取干预措施以防止高热的严重后果。