El Ghonemi Mohamed, Omar Hesham R, Rashad Rania, Kolla Jaya, Mangar Devanand, Camporesi Enrico
Department of Critical Care, Cairo University, Cairo, Egypt.
Cases J. 2009 Dec 29;2:9402. doi: 10.1186/1757-1626-2-9402.
Toxic Epidermal Necrolysis and Steven-Johnson syndrome are entities on a spectrum of cutaneous reactions that usually occur as an idiosyncratic reaction to certain drugs. The distinction between TEN and SJS is based on the percentage of skin involved with SJS being less than 10% and TEN being more than 30%. They exhibit severe skin blistering and sloughing with mucosal involvement and can be fatal in many cases. Discontinuation of the offending agent is mandatory together with reduction of skin manipulation and avoiding infection. Plasmapharesis, intravenous immunoglobulins and immunosuppressants have been used with conflicting results. In this manuscript we are describing a 22 year old female patient from Egypt who presented with severe skin sloughing with mucosal involvement following carbamazepine therapy. The incriminated drug was discontinued and urgent life saving therapy in the form of broad spectrum antibiotic, immunosuppression with cyclophosphamide, Intensive Care Unit admission and nursing care was started followed by dramatic response. The clinical presentation, pathogenesis and modalities of treatment will be described in details.
中毒性表皮坏死松解症和史蒂文斯-约翰逊综合征是一系列皮肤反应中的病症，通常作为对某些药物的特异反应而出现。中毒性表皮坏死松解症（TEN）和史蒂文斯-约翰逊综合征（SJS）的区别基于皮肤受累的百分比，SJS小于10%，TEN大于30%。它们表现为严重的皮肤水疱和脱落，并伴有黏膜受累，在许多情况下可能致命。必须停用致病药物，同时减少皮肤操作并避免感染。血浆置换、静脉注射免疫球蛋白和免疫抑制剂的使用结果相互矛盾。在本手稿中，我们描述了一名来自埃及的22岁女性患者，她在服用卡马西平治疗后出现严重的皮肤脱落并伴有黏膜受累。停用了涉案药物，并开始了以广谱抗生素、环磷酰胺免疫抑制、入住重症监护病房和护理为形式的紧急挽救生命治疗，随后出现显著反应。将详细描述临床表现、发病机制和治疗方式。
