在这里,我们报道了一个12岁女性的病例,她注意到最近出现了椭圆形,受限制,影响大腿和外阴的10厘米乳头状瘤斑块显示淋巴水肿的组织学征象,没有继发性淋巴水肿的证据。与淋巴水肿或表皮痣(EN)延迟发作相关的基因测序-GATA2和GJC2以及HRAS和KRAS,分别-显示野生型等位基因。人乳头瘤病毒(HPV)DNA的聚合酶链反应证明了15种HPV基因型的感染。生产性HPV感染的证据,HPV衣壳表达,检测到细胞病变。在6个月的随访中,完全切除后没有发现复发的证据.对一系列连续的91个EN切除标本的分析表明,有76%的人表现出淋巴停滞的组织学证据。值得注意的是,多个acrochorddon-likeEN,最类似于这种情况,有类似的局部淋巴水肿征象.淋巴水肿的迟发和证据有利于先天性单节淋巴水肿的诊断。然而,临床表现和表皮改变提示EN的诊断。此外,局部疣状病变也能准确描述患者的皮肤表现。根据上述证据,我们推测淋巴管的异常发育可能在某些类型EN的发病机制中起主要作用,并促进生产性HPV感染.
Herein, we report the case of a 12-year-old female who noted the recent onset of an oval, circumscribed, 10-cm papillomatous plaque affecting the thigh and vulva that showed histologic signs of lymphedema without evidence of secondary lymphedema. The sequencing of genes associated with a delayed onset of lymphedema or epidermal nevi (EN) - GATA2 and GJC2, and HRAS and KRAS, respectively - showed wild-type alleles. Polymerase chain reaction for human papillomavirus (HPV) DNA demonstrated infections with 15 HPV genotypes. Evidence of productive HPV infection, HPV capsid expression, and cytopathic changes was detected. At the 6-month follow-up, no evidence of recurrence was found after complete excision. The analysis of a consecutive series of 91 EN excision specimens revealed that 76% exhibited histologic evidence of lymphostasis. Notably, multiple acrochordon-like EN, which most closely resembled this case, showed similar signs of localized lymphedema. The late onset and evidence of lymphedema favors the diagnosis of congenital unisegmental lymphedema. However, the clinical findings and epidermal changes point to the diagnosis of EN. Moreover, localized verrucosis also accurately describes this patient\'s cutaneous findings. Based on the above evidence, we postulate that an abnormal development of lymphatics may play a primary role in the pathogenesis of some types of EN and facilitate productive HPV infection.