Sekar Nishanth, Umakanth Maheswaran
Internal Medicine, Faculty of Health Care Sciences, The Eastern University, Chenkalady, LKA.
Clinical Medicine, Teaching Hospital Batticaloa, Batticaloa, LKA.
Cureus. 2022 Mar 28;14(3):e23580. doi: 10.7759/cureus.23580. eCollection 2022 Mar.
Idiopathic hypereosinophilic syndrome is a rare entity where the diagnosis is made after all the probable causes of hypereosinophilia are excluded. The characteristic organ involvement includes the heart, nervous system, lung, and gastrointestinal tract. The mainstay of treatment is corticosteroids. Patients who are unresponsive to the steroids require immunomodulatory therapy that includes imatinib, mepolizumab, and in some resistant cases alemtuzumab. We describe a case of idiopathic hypereosinophilic syndrome with skin involvement without other organ infiltration in a previously unevaluated South Asian male who responded well to the initiation of steroid therapy.
特发性嗜酸性粒细胞增多综合征是一种罕见的病症,在排除所有可能导致嗜酸性粒细胞增多的原因后才能做出诊断。其特征性的器官受累包括心脏、神经系统、肺和胃肠道。治疗的主要手段是使用皮质类固醇。对类固醇无反应的患者需要免疫调节治疗,包括伊马替尼、美泊利单抗,在一些耐药病例中还需要使用阿仑单抗。我们描述了一例特发性嗜酸性粒细胞增多综合征病例,该病例发生在一名之前未接受评估的南亚男性身上,有皮肤受累但无其他器官浸润,对开始的类固醇治疗反应良好。