Oliveira Ana Maria, Carvalho Rita, Martins Alexandra, Reis Jorge
Gastroenterology Department, Hospital Prof. Doutor Fernando Fonseca, Amadora, Portugal.
GE Port J Gastroenterol. 2016 Aug 12;23(6):304-308. doi: 10.1016/j.jpge.2016.06.001. eCollection 2016 Nov-Dec.
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe, idiosyncratic reaction characterized by diffuse maculopapular rash, facial edema, lymphadenopathy, fever, eosinophilia and/or other leukocyte abnormalities, and involvement of internal organs as liver, kidney, heart and lung. Diagnosing this entity is specifically complicated due to the multiplicity of organs involved. DRESS syndrome must be recognized promptly and the causative drug withdrawn in order to improve patient outcomes. Indeed, it is a potentially life-threatening condition, with a reported mortality between 5 and 20%. We describe a case of a 22-year old woman admitted to our hospital with acute diffuse, pruritic rash associated with crampy abdominal pain, vomiting, diarrhea and fever three weeks after starting sulfasalazine therapy. Initially, laboratory parameters revealed normal white blood cell count and normal liver enzymes, but during hospitalization, eosinophilia developed and liver enzymes, including transaminases and cholestatic parameters, dramatically increased. The diagnostic of DRESS syndrome was made and sulfasalazine was withdrawn and as there were signs of disease severity, systemic corticotherapy was initiated, with gradually improvement of the rash and symptoms resolution. The patient was discharged home after thirty days of hospitalization.
药物超敏反应伴嗜酸性粒细胞增多和全身症状(DRESS)综合征是一种严重的特异性反应,其特征为弥漫性斑丘疹、面部水肿、淋巴结病、发热、嗜酸性粒细胞增多和/或其他白细胞异常,以及肝脏、肾脏、心脏和肺等内脏器官受累。由于涉及多个器官,诊断这一病症特别复杂。必须迅速识别DRESS综合征并停用致病药物,以改善患者预后。事实上,这是一种潜在的危及生命的病症,据报道死亡率在5%至20%之间。我们描述了一例22岁女性患者,在开始使用柳氮磺胺吡啶治疗三周后,因急性弥漫性瘙痒性皮疹伴痉挛性腹痛、呕吐、腹泻和发热入住我院。最初,实验室检查参数显示白细胞计数和肝酶正常,但住院期间出现了嗜酸性粒细胞增多,包括转氨酶和胆汁淤积参数在内的肝酶显著升高。诊断为DRESS综合征,停用柳氮磺胺吡啶,由于有疾病严重的迹象,开始进行全身皮质激素治疗,皮疹逐渐改善,症状消退。患者住院30天后出院回家。