3例Netherton综合征患者中局部用他克莫司出现显著吸收。
Significant absorption of topical tacrolimus in 3 patients with Netherton syndrome.
作者信息
Allen A, Siegfried E, Silverman R, Williams M L, Elias P M, Szabo S K, Korman N J
机构信息
Department of Dermatology, Saint Louis University Health Sciences Center, 1755 S Grand, St Louis, MO 63104, USA.
出版信息
Arch Dermatol. 2001 Jun;137(6):747-50.
BACKGROUND
Tacrolimus is a macrolide immunosuppressant approved in oral and intravenous formulations for primary immunosuppression in liver and kidney transplantation. Topical 0.1% tacrolimus ointment has recently been shown to be effective in atopic dermatitis for children as young as 2 years of age, with minimal systemic absorption. We describe 3 patients treated with topical 0.1% tacrolimus who developed significant systemic absorption.
OBSERVATION
Three patients previously diagnosed as having Netherton syndrome were treated at different centers with 0.1% tacrolimus ointment twice daily. Two patients showed dramatic improvement. All patients were found to have tacrolimus blood levels within or above the established therapeutic trough range for oral tacrolimus in organ transplant recipients. None of these patients developed signs or symptoms of toxic effects of tacrolimus.
CONCLUSIONS
Patients with Netherton syndrome have a skin barrier dysfunction that puts them at risk for increased percutaneous absorption. The Food and Drug Administration recently approved 0.1% tacrolimus ointment for the treatment of atopic dermatitis. Children with Netherton syndrome may be misdiagnosed as having atopic dermatitis. These children are at risk for marked systemic absorption and associated toxic effects. If topical tacrolimus is used in this setting, monitoring of serum tacrolimus levels is essential.
背景
他克莫司是一种大环内酯类免疫抑制剂,有口服和静脉注射两种剂型,已被批准用于肝、肾移植的初始免疫抑制治疗。最近研究表明,局部使用0.1%他克莫司软膏对年仅2岁的儿童特应性皮炎有效,且全身吸收极少。我们报道了3例使用局部0.1%他克莫司治疗后出现显著全身吸收的患者。
观察
3例先前诊断为Netherton综合征的患者在不同中心接受每日2次的0.1%他克莫司软膏治疗。2例患者病情显著改善。所有患者的他克莫司血药浓度均在器官移植受者口服他克莫司既定治疗谷浓度范围内或高于该范围。这些患者均未出现他克莫司中毒的体征或症状。
结论
Netherton综合征患者存在皮肤屏障功能障碍,使其经皮吸收增加。美国食品药品监督管理局最近批准0.1%他克莫司软膏用于治疗特应性皮炎。患有Netherton综合征的儿童可能会被误诊为特应性皮炎。这些儿童有显著全身吸收及相关毒性作用的风险。若在此情况下使用局部他克莫司,监测血清他克莫司水平至关重要。