Downey Camila, Requena Luis, Bagué Silvia, Sánchez Martínez Miquel Ángel, Lloreta Josep, Baselga Eulalia
Department of Dermatology, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Department of Dermatology, Fundación Jiménez Díaz, Madrid, Spain.
Pediatr Dermatol. 2016 Jul;33(4):e240-3. doi: 10.1111/pde.12896. Epub 2016 Jun 14.
Connective tissue nevi are benign hamartomatous lesions in which one or several of the components of the dermis (collagen, elastin, glicosaminoglycans) show predominance or depletion. Recently, de Feraudy et al broadened the spectrum of connective tissue nevus, describing fibroblastic connective tissue nevus (FCTN), which is characterized by proliferation of CD34(+) cells of fibroblastic and myofibroblastic lineage. Only solitary papules and nodules have been described. We present the first case of FCTN with multiple agminated lesions on the leg of an infant and the difficulties encountered in the differential diagnosis with dermatofibrosarcoma protuberans.
结缔组织痣是一种良性错构瘤性病变，其中真皮的一种或几种成分（胶原蛋白、弹性蛋白、糖胺聚糖）显示为主导或减少。最近，德费劳迪等人拓宽了结缔组织痣的范围，描述了成纤维细胞性结缔组织痣（FCTN），其特征是成纤维细胞和肌成纤维细胞谱系的CD34(+)细胞增殖。此前仅描述过孤立性丘疹和结节。我们报告了首例发生于婴儿腿部的具有多个聚集性病变的FCTN病例，以及在与隆突性皮肤纤维肉瘤进行鉴别诊断时遇到的困难。
