1型神经纤维瘤病相关的下肢皮肤溃疡：一例报告
Lower Extremity Skin Ulcer Associated With Neurofibromatosis Type 1: A Case Report.
作者信息
Gracia-Cazaña Tamara, de la Fuente Sonia, Concellón María A, Ara-Martín Mariano
机构信息
Department of Dermatology, Hospital de Barbastro, Huesca, Spain.
Department of Dermatology, Hospital Ernest Lluch, Calatayud, Zaragoza, Spain.
出版信息
Wounds. 2019 Oct;31(10):E65-E67.
INTRODUCTION
The association between neurofibromatosis type 1 (NF-1) and vasculopathy has been reported frequently, especially cerebral, intestinal, and peripheral vasculopathy. However, cutaneous vasculopathy is infrequent.
CASE REPORT
The authors present the case of a 32-year-old man with a painful ulcer on his left thigh of 3 weeks' duration in the same location as a long-time capillary malformation associated with alopecia. The skin biopsy showed signs of perivascular fibromuscular dysplasia with proliferating myofibroblastic cells. The patient had been treated with various therapeutic options, such as topical antibiotics, oral and intralesional corticosteroids, and oral cyclosporine and intravenous prostanoids.
CONCLUSIONS
Cutaneous vasculopathy related to the skin, such as livedo reticularis and ulcers of torpid evolution due to cutaneous vasculopathy are extremely rare. Thus, it is necessary to include skin ulcers as one of the phenotypic manifestations of NF-1.
引言
1型神经纤维瘤病（NF-1）与血管病变之间的关联已被频繁报道，尤其是脑部、肠道和周围血管病变。然而，皮肤血管病变并不常见。
病例报告
作者报告了一例32岁男性患者，其左大腿出现一处持续3周的疼痛性溃疡，溃疡位于与长期存在的伴有脱发的毛细血管畸形相同的位置。皮肤活检显示血管周围纤维肌发育异常，伴有肌成纤维细胞增生。该患者接受了多种治疗方案，如局部抗生素、口服和病灶内注射皮质类固醇、口服环孢素和静脉注射前列腺素。
结论
与皮肤相关的皮肤血管病变，如网状青斑和因皮肤血管病变导致的慢性进展性溃疡极为罕见。因此，有必要将皮肤溃疡纳入NF-1的表型表现之一。
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