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白塞病合并皮肤白细胞破碎性血管炎。对泼尼松和苯丁酸氮芥的反应。

Behçet's syndrome complicated by cutaneous leukocytoclastic vasculitis. Response to prednisone and chlorambucil.

作者信息

Plotkin G R, Patel B R, Shah V N

出版信息

Arch Intern Med. 1985 Oct;145(10):1913-5.

PMID:4037953
Abstract

Traditionally described as a triad consisting of recurrent aphthous stomatitis, genital ulcerations, and uveitis, Behçet's syndrome is now recognized as a multisystem disease with protean manifestations. We studied a patient with chronic recurrent migratory superficial thrombophlebitis and marked cutaneous hyperreactivity (pathergy) who developed leukocytoclastic vasculitis with recalcitrant leg ulcerations nine years after the onset of his illness. Although he was treated with topical and systemic antibiotics for presumed bacterial superinfection, cutaneous ulcerations continued to develop and enlarge; only after prednisone therapy was begun was there a dramatic response with complete resolution. Despite high-dose prednisone therapy, the pathergy test remained markedly positive; however, no new skin ulcerations appeared.

摘要

白塞病传统上被描述为由复发性口腔溃疡、生殖器溃疡和葡萄膜炎组成的三联征,现在被认为是一种具有多种表现的多系统疾病。我们研究了一名患有慢性复发性游走性浅表血栓性静脉炎和明显皮肤高反应性(同形反应)的患者,该患者在发病九年后出现了白细胞破碎性血管炎,并伴有顽固性腿部溃疡。尽管因推测存在细菌重叠感染而接受了局部和全身抗生素治疗,但皮肤溃疡仍持续发展和扩大;仅在开始使用泼尼松治疗后才出现显著反应,溃疡完全愈合。尽管使用了高剂量泼尼松治疗,同形反应试验仍显著阳性;然而,未出现新的皮肤溃疡。

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