Panda Prasan Kumar, Prajapati Ramjas, Kumar Arvind, Jana Manisha, Immanuel Pradeep, Tanwar Pranay, Wig Naveet
Department of Medicine, All India Institute of Medical Sciences, Delhi, India.
Department of Radiology, All India Institute of Medical Sciences, Delhi, India.
Intractable Rare Dis Res. 2017 Aug;6(3):230-233. doi: 10.5582/irdr.2017.01048.
A young adult man with 4-years history of lepromatous leprosy (received irregularly multidrug therapy) presented with two and half years history of symptoms suggestive of chronic erythema nodosum leprosum (ENL), initially responded to steroids and thalidomide, but later on failed. During the last 2-months, he developed fever, vomiting, and subsequently altered sensorium. On evaluation, he had hepatosplenomegaly, hyponatremia, hyperferritinemia, hemophagocytosis in bone marrow aspiration, lobular panniculitis in skin biopsy, and multiple parenchymal nodules in chest imaging. Hence diagnosis of ENL with hemophagocytic lymphohistiocytic (HLH) syndrome was established and treatment with dexamethasone (10 mg/m) started. During hospitalization, he developed sinus bradycardia, QT prolongations, recurrent ventricular tachycardia, and moderate systolic dysfunction. The cardiac complications recovered using a temporary pacemaker and were presumed to be due to micronodular cardiac deposition of ENL. This case iterates that ENL can present with varied presentations like asymptomatic lung nodules and storming cardiac complications. More importantly leprosy, ENL, and HLH are a continuum of manifestations of the same agent-host interactions.
一名患有4年瘤型麻风病史(接受过不规则多药治疗)的年轻成年男性,出现了两年半提示慢性结节性红斑麻风(ENL)的症状,最初对类固醇和沙利度胺有反应,但后来治疗失败。在过去的两个月里,他出现了发热、呕吐,随后意识改变。经评估,他有肝脾肿大、低钠血症、高铁蛋白血症、骨髓穿刺显示噬血细胞现象、皮肤活检显示小叶性脂膜炎以及胸部影像学显示多个实质结节。因此,确诊为伴有噬血细胞性淋巴组织细胞增生症(HLH)综合征的ENL,并开始用地塞米松(10mg/m)治疗。住院期间,他出现了窦性心动过缓、QT间期延长、反复发作的室性心动过速以及中度收缩功能障碍。使用临时起搏器后心脏并发症得以恢复,推测是由于ENL的微结节性心脏沉积所致。该病例表明,ENL可表现为多种形式,如无症状肺结节和突发心脏并发症。更重要的是,麻风、ENL和HLH是同一病原体与宿主相互作用的连续表现形式。