Acta Chir Plast. 2020 Winter;62(3-4):111-114.
Extramammary vulvar Paget's disease is a very rare presentation of this disease, with few symptoms, whose initial complaint is the appearance of a slow-growing erythematous plaque in the anogenital region associated with pruritus. The evolution is chronic and the diagnosis is often late. Surgical excision is the main treatment and should be performed with wide margins, due to the high rate of local recurrence. Reconstruction is often complex, requiring the use of local or remote flaps. This manuscript presents the case report of a 65-year-old female patient, who had been suffering from pruritus in the vulvar region for 2 years and had a well-defined erythematous lesion, which showed no improvement with topical treatments and that was diagnosed as Paget's disease after biopsy. After surgical excision, reconstruction was performed using bilateral myofasciocutaneous flap of the gracilis muscle, with excellent aesthetic and functional results..
外阴派杰氏病是一种非常罕见的疾病表现，症状较少，其最初的表现为肛门生殖器区域出现缓慢生长的红斑斑块，并伴有瘙痒。疾病的发展是慢性的，诊断往往较晚。手术切除是主要的治疗方法，由于局部复发率高，应进行广泛的边缘切除。重建通常很复杂，需要使用局部或远程皮瓣。本文报告了一例 65 岁女性患者，她患有外阴瘙痒 2 年，外阴有一个界限清楚的红斑性病变，局部外用治疗无效，经活检诊断为派杰氏病。手术后，采用双侧股薄肌的肌筋膜皮瓣进行重建，取得了良好的美学和功能效果。
