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慢性腿部溃疡作为营养不良性大疱性表皮松解症首次诊断的罕见病因。

Chronic leg ulcers as a rare cause for the first diagnosis of epidermolysis bullosa dystrophica.

作者信息

Bafaraj Mazin G, Cesko Elvir, Weindorf Maren, Dissemond Joachim

机构信息

Department of Dermatology, University Hospital of Essen, Essen, Germany.

出版信息

Int Wound J. 2014 Jun;11(3):274-7. doi: 10.1111/j.1742-481X.2012.01087.x. Epub 2012 Sep 13.

Abstract

Chronic leg ulcers occur most frequently in the elderly population as a result of an underlying vascular disease especially chronic venous insufficiency. But it also occurs less commonly in younger people due to other aetiologies, for example, infections, vasculitis, neoplasia or genetic diseases. The following case report presents chronic leg ulcers as a rare cause for the first diagnosis of dystrophic epidermolysis bullosa. We report about a 21-year-old man with painful chronic leg ulcers resistant to different wound treatments for 4 months. After exclusion of the more common vascular aetiologies and reviewing the patient's family history, we considered an epidermolysis bullosa dystrophica which could be confirmed by genetic analyses. We treated the patient with debridement, modified negative pressure therapy with non-adhesive foil and skin grafting. The chronic leg ulcers healed completely. This case report demonstrates that the family history and genetic diseases should be considered as rare causes for therapy-refractory chronic leg ulcers, especially in young patients.

摘要

慢性腿部溃疡在老年人群中最为常见,是由潜在的血管疾病尤其是慢性静脉功能不全引起的。但由于其他病因,如感染、血管炎、肿瘤或遗传性疾病,在年轻人中也较少见。以下病例报告介绍了慢性腿部溃疡作为营养不良性大疱性表皮松解症首次诊断的罕见原因。我们报告了一名21岁男性,患有疼痛性慢性腿部溃疡,对不同伤口治疗方法均无效,持续了4个月。在排除了更常见的血管病因并查阅患者家族史后,我们考虑为营养不良性大疱性表皮松解症,这可通过基因分析得到证实。我们对患者进行了清创、使用非粘性箔片的改良负压治疗和皮肤移植。慢性腿部溃疡完全愈合。本病例报告表明,家族史和遗传性疾病应被视为治疗难治性慢性腿部溃疡的罕见原因,尤其是在年轻患者中。

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