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[莱尔综合征后的眼部后遗症和干燥综合征]

[Ocular sequelae and sicca syndrome following Lyell's syndrome].

作者信息

Roujeau J C, Phlippoteau C, Koso M, Wechsler J, Binaghi M, Revuz J, Touraine R

出版信息

Ann Dermatol Venereol. 1985;112(11):883-8.

PMID:3879142
Abstract

The mucous lesions are customary in toxic epidermal necrolysis. The acute ocular lesions have led to definitive sequelae. 22 out of 32 patients surviving to a Lyell's syndrome lend themselves to an ophthalmological supervision. 13 of these 22 patients (59 p. 100) presented a sicca syndrome with a decrease of the lacrimal secretion (12 cases) and/or salivary secretion (8 cases). The sicca syndrome appeared sometimes from the beginning of the acute phase of the Lyell's syndrome or more often a few weeks later. This decrease of the lacrimal flux went along with objective corneal lesions among 11 patients, and 6 of them had a change of the visual function. A biopsy of the accessory salivary glands was performed on 7 of these patients having a reduction of the salivary flux. 5 of these 7 biopsies showed lymphocytic infiltrates giving in 2 cases a nodular aspect, grade III of Chisholm's classification, held to be pathognomonic of Sjögren syndrome. The 13 patients having presented a sicca syndrome differed from the other 9 patients (without sicca syndrome) by a higher severity of their Lyell's syndrome and by a higher age. None of our patients with dry syndrome had antinuclear antibody detected by immunofluorescence on sections of rat's livers. Sicca syndromes with sometimes lymphocytic infiltrate similar to those of Sjögren's syndrome were occasionally imputed to drug reactions. The occurrence of "Sjögren like's syndrome" after a toxic epidermal necrolysis suggests the intervening of autoimmune phenomena in the pathogenesis of this toxic epidermal necrolysis.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

黏液性病变在中毒性表皮坏死松解症中很常见。急性眼部病变已导致明确的后遗症。32例存活至Lyell综合征的患者中有22例需要眼科监护。这22例患者中有13例(占59%)出现干燥综合征,泪液分泌减少(12例)和/或唾液分泌减少(8例)。干燥综合征有时在Lyell综合征急性期开始时出现,或更常见于几周后。11例患者泪液流量减少伴有客观的角膜病变,其中6例视力功能发生改变。对7例唾液流量减少的患者进行了副唾液腺活检。这7例活检中有5例显示淋巴细胞浸润,2例呈结节状,为Chisholm分类的III级,被认为是干燥综合征的特征性表现。出现干燥综合征的13例患者与其他9例(无干燥综合征)患者的不同之处在于,他们的Lyell综合征病情更严重,年龄更大。我们的干燥综合征患者在大鼠肝脏切片免疫荧光检查中均未检测到抗核抗体。有时与干燥综合征类似的淋巴细胞浸润的干燥综合征偶尔被归因于药物反应。中毒性表皮坏死松解症后出现“类干燥综合征”提示自身免疫现象参与了这种中毒性表皮坏死松解症的发病机制。(摘要截选至250字)

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