Taillia H, Alla P, Fournier B, Bounolleau P, Ouologem M, Ricard D, Sallansonnet-Froment M, de Greslan T, Renard J-L
hôpital d'instruction des armées du Val-de-Grâce, Paris, France.
Rev Neurol (Paris). 2009 Oct;165(10):821-7. doi: 10.1016/j.neurol.2009.02.009. Epub 2009 Apr 11.
Anticonvulsant hypersensitivity syndrome (AHS) is defined by the association of high fever, cutaneous rash and multiorgan-system abnormalities (incidence, one in 1000 to one in 10,000 exposures). Fatal complications are described in 10%. This reaction usually develops 1 to 12 weeks after initiation of an aromatic anticonvulsant. Drug rash with eosinophilia and systemic symptoms (DRESS) can be discussed as differential diagnosis. Several hypotheses have been put forward to explain the pathogenesis of AHS. These include accumulation of toxic metabolites, antibody production and viral infection. The one based on toxic metabolites has found the greatest acceptance due to the fact that it can be proven by an in vitro test, the lymphocyte toxicity assay. In vivo, skin biopsies show characteristic findings of erythema multiform or typical leucocytoclastic angitis. The patch-test is positive in 80% of the cases. Lamotrigine-associated anticonvulsant hypersensitivity syndrome (LASH) is rare and was described in 1998. We report two new cases demonstrating the two particular configurations of apparition of LASH found in the 14 cases from the review of literature (Pubmed: anticonvulsant hypersensitivity syndrome - lamotrigine): high doses of lamotrigine (or lamotrigine in very young or old patients), and lamotrigine associated with another anti-epileptic (phenobarbital or sodium valproate). We discuss the links between DRESS after lamotrigine and LASH as illustrated in a new case.
抗惊厥药超敏反应综合征(AHS)的定义为高热、皮疹和多器官系统异常同时出现(发病率为每1000至10000次用药中有1例)。10%的病例会出现致命并发症。这种反应通常在开始使用芳香族抗惊厥药后1至12周出现。药物性皮疹伴嗜酸性粒细胞增多和全身症状(DRESS)可作为鉴别诊断进行讨论。已经提出了几种假说来解释AHS的发病机制。这些假说包括有毒代谢产物的积累、抗体产生和病毒感染。基于有毒代谢产物的假说得到了最广泛的认可,因为它可以通过体外试验淋巴细胞毒性测定得到证实。在体内,皮肤活检显示多形红斑或典型白细胞破碎性血管炎的特征性表现。斑贴试验在80%的病例中呈阳性。拉莫三嗪相关的抗惊厥药超敏反应综合征(LASH)很罕见,于1998年被描述。我们报告了两例新病例,展示了在文献回顾(PubMed:抗惊厥药超敏反应综合征 - 拉莫三嗪)中14例病例中发现的LASH出现的两种特殊情况:高剂量拉莫三嗪(或非常年幼或年老患者使用的拉莫三嗪),以及拉莫三嗪与另一种抗癫痫药物(苯巴比妥或丙戊酸钠)联用。我们讨论了拉莫三嗪后发生DRESS与LASH之间的联系,如在一个新病例中所示。