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莱尔综合征。

Lyell's syndrome.

作者信息

Baroni Adone, Ruocco Eleonora

机构信息

Department of Dermatology, Second University of Naples, Naples, Italy.

出版信息

Skinmed. 2005 Jul-Aug;4(4):221-5. doi: 10.1111/j.1540-9740.2005.03593.x.

Abstract

Lyell's syndrome, or toxic epidermal necrolysis, is a rare, potentially life-threatening mucocutaneous disease, usually provoked by the administration of a drug and characterized by acute necrosis of the epidermis. The drugs most frequently incriminated are nonsteroidal anti-inflammatory drugs, chemotherapics, antibiotics, and anticonvulsants. An immunologic response to immunocomplexes formed by metabolites of the causal drug and the common tissue antigens is thought to be responsible for this disorder. Preceded by fever, general malaise, and other flu-like symptoms, bullous and erosive lesions involve oral, ocular, and genital mucosae; and vast areas of the skin with extensive dermoepidermal detachments. The loss of fluid and electrolytes and supervening infections lead to a severe general condition, often with fatal outcome. Treatment is based on the administration of fluids, electrolytes, and albumin. The use of systemic corticosteroids is controversial. Plasmapheresis and hyperbaric oxygen proved to be useful. The employment of high doses of IV immunoglobulins is a novel, valid, and promising treatment.

摘要

莱尔综合征,即中毒性表皮坏死松解症,是一种罕见的、可能危及生命的皮肤黏膜疾病,通常由药物使用引发,其特征为表皮急性坏死。最常涉及的药物是非甾体抗炎药、化疗药物、抗生素和抗惊厥药。对由致病药物代谢产物与常见组织抗原形成的免疫复合物产生的免疫反应被认为是该疾病的病因。在发热、全身不适及其他类似流感症状之后,出现水疱和糜烂性病变,累及口腔、眼部和生殖器黏膜;以及大面积皮肤出现广泛的皮肤表皮分离。体液和电解质流失以及随后发生的感染导致严重的全身状况,常以死亡告终。治疗基于补充液体、电解质和白蛋白。全身使用皮质类固醇存在争议。血浆置换和高压氧治疗已被证明有效。大剂量静脉注射免疫球蛋白的应用是一种新颖、有效且有前景的治疗方法。

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