de Campos Fernando Peixoto Ferraz, de Lima Patricia Picciarelli, Maragno Luciana, Watanabe Fabio Toshio
Department of Internal Medicine - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
Autops Case Rep. 2012 Dec 31;2(4):5-14. doi: 10.4322/acr.2012.029. eCollection 2012 Oct-Dec.
Drug-induced hypersensitivity syndrome (DIHS; also known as drug reaction with eosinophilia and systemic symptoms [DRESS]) is a life-threatening condition first described by Chaiken et al. in 1950. It is characterized by extensive mucocutaneous rash; fever; lymphadenopathy; hepatitis; hematological abnormalities; damage to several organs such as kidney, heart, lungs, and pancreas; and possible reactivation of human herpesvirus-6 (HHV-6) or other herpes virus. Rare and severe cases may present hepatic necrosis, and about 15% of them result in death or liver transplantation. A hallmark of this syndrome is the late onset of symptoms after the drug exposure. The most common culprit drugs are the aromatic anticonvulsants (in almost 30% of the cases) and the antibiotics (which in some series represent 20% of the cases). The authors report a case of a 41-year-old female who presented to the emergency department with erythroderma, acute hepatitis, acute pancreatitis and acute renal failure, and was then treated with corticosteroid after the diagnosis of DIHS/DRESS. A specific culprit drug could not confidently be determined due to the presence of multiple drugs used by the patient. The clinical and laboratory outcome was apparently satisfactory, but unexpectedly, on the sixth day of hospitalization, the patient complained of nonspecific malaise, drowsiness, which progressed in a few hours with signs and symptoms of hepatic failure, refractory shock, and death. The autopsy findings showed submassive hepatic necrosis, and the immediate cause of death was attributed to hepatic failure.
药物性超敏反应综合征(DIHS;也称为伴有嗜酸性粒细胞增多和全身症状的药物反应[DRESS])是一种危及生命的疾病,最早由柴肯等人于1950年描述。其特征为广泛的黏膜皮肤皮疹、发热、淋巴结病、肝炎、血液学异常、对肾脏、心脏、肺和胰腺等多个器官的损害,以及人疱疹病毒6型(HHV - 6)或其他疱疹病毒的可能重新激活。罕见且严重的病例可能出现肝坏死,其中约15%会导致死亡或肝移植。该综合征的一个标志是药物暴露后症状出现较晚。最常见的致病药物是芳香族抗惊厥药(几乎占病例的30%)和抗生素(在某些系列中占病例的20%)。作者报告了一例41岁女性病例,该患者因红皮病、急性肝炎、急性胰腺炎和急性肾衰竭就诊于急诊科,在诊断为DIHS/DRESS后接受了皮质类固醇治疗。由于患者使用了多种药物,无法确切确定具体的致病药物。临床和实验室结果显然令人满意,但出乎意料的是,在住院第六天,患者抱怨有非特异性不适、嗜睡,数小时内病情进展,出现肝衰竭、难治性休克的体征和症状,最终死亡。尸检结果显示亚大块肝坏死,直接死因归因于肝衰竭。