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[伴有皮肤症状的肝性卟啉病]

[Hepatic porphyrias with cutaneous symptoms].

作者信息

Timonen Kaisa, Nuutinen Pauliina, Raili Kauppinen

出版信息

Duodecim. 2012;128(12):1247-55.

Abstract

Hepatic porphyrias with cutaneous symptoms Cutaneous symptoms of porphyrias are initiated from a phototoxic reaction caused by sunlight and circulating porphyrins in the vascular walls of the skin. This leads in fragility, blistering and scarring of the skin on light-exposed areas. There are approximately 200 patients having hepatic porphyrias with cutaneous symptoms in Finland. Cutaneous symptoms of variegate porphyria and porphyria cutanea tarda are indistinguishable, but an effective treatment is available only for the latter. Differential diagnosis is important due to acute episodes occurring in variegate porphyria.

摘要

伴有皮肤症状的肝性卟啉病 卟啉病的皮肤症状由阳光及皮肤血管壁中循环的卟啉引发的光毒性反应所致。这会导致暴露于光的部位皮肤出现脆弱、水疱及瘢痕形成。芬兰约有200例伴有皮肤症状的肝性卟啉病患者。混合型卟啉病和迟发性皮肤卟啉病的皮肤症状难以区分,但仅对后者有有效的治疗方法。由于混合型卟啉病会出现急性发作,鉴别诊断很重要。

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