Bobot Mickaël, Coen Matteo, Simon Clémentine, Daniel Laurent, Habib Gilbert, Serratrice Jacques
Service of Nephrology and Renal Transplantation, La Conception University Hospital, Boulevard Baille, Marseille, France Department of Internal Medicine, Rehabilitation and Geriatrics, Service of Internal Medicine, Geneva University Hospitals, rue Gabrielle Perret-Gentil, Geneva, Switzerland Department of Pathology and Immunology, rue Michel Servet, Geneva, Switzerland Department of Radiology Department of Pathology Department of Cardiology, La Timone University Hospital, rue Saint Pierre Marseille, France.
Medicine (Baltimore). 2018 Apr;97(15):e0297. doi: 10.1097/MD.0000000000010297.
The life-threatening drug rash with eosinophilia and systemic symptoms (DRESS) syndrome occurs most commonly after exposure to drugs, clinical features mimic those found with other serious systemic disorders. It is rarely associated with thrombotic microangiopathy.
We describe the unique case of a 44-year-old man who simultaneously experienced DRESS syndrome with thrombotic microangiopathy (TMA) after a 5 days treatment with fluindione.
Clinical evaluation leads to the discovery of an underlying lymphangiomatosis, due to a Noonan syndrome.
The anticoagulant was withdrawn, and corticosteroids (1 mg/kg/day) and acenocoumarol were started.
Clinical improvement ensued. At follow-up the patient is well.
The association of DRESS with TMA is a rare condition; we believe that the presence of the underlying Noonan syndrome could have been the trigger. Moreover, we speculate about the potential interrelations between these entities.
危及生命的药物疹伴嗜酸性粒细胞增多和全身症状（DRESS）综合征最常发生于接触药物后，其临床特征与其他严重全身性疾病相似。它很少与血栓性微血管病相关。
我们描述了一名44岁男性的独特病例，该患者在接受氟茚二酮治疗5天后同时出现了伴有血栓性微血管病（TMA）的DRESS综合征。
临床评估发现潜在的淋巴管瘤病，病因是努南综合征。
停用抗凝剂，开始使用皮质类固醇（1毫克/千克/天）和醋硝香豆素。
临床症状改善。随访时患者情况良好。
DRESS与TMA的关联是一种罕见情况；我们认为潜在的努南综合征可能是触发因素。此外，我们推测了这些病症之间的潜在相互关系。
