Wu Yu-Hung, Tu Mei-Eng, Lee Chyou-Shen, Lin Yang-Chih
Department of Dermatology, Mackay Memorial Hospital, Taipei, Taiwan.
J Cutan Pathol. 2009 Mar;36(3):355-8. doi: 10.1111/j.1600-0560.2008.01037.x.
Necrolytic acral erythema is a newly described entity characterized by sharply demarcated scaly plaques on the dorsum of the hands and feet. More than 30 patients have been reported since 1996, all of whom had anti-hepatitis C virus antibody. A 32-year-old Taiwanese woman had been diagnosed with and treated for systemic lupus erythematosus with lupus nephritis about 10 years earlier. Soon thereafter, she noted several well-demarcated keratotic plaques with erythematous borders on her feet, with sparing of the soles. Histopathology showed diffuse parakeratosis with a neutrophil infiltrate, hypogranulosis, pale upper keratinocytes, scattered and grouped dyskeratotic cells, psoriasiform hyperplasia and a mild lymphocytic infiltrate in the upper dermis. The diagnosis was made after three biopsies. The lesions regularly worsened just before and during menstruation, but patch and intradermal tests for progesterone and estrogen were negative. There was no evidence of either hepatitis B or hepatitis C infection. The lesions did not respond to treatment with zinc. The rash regressed spontaneously when corticosteroids were stopped and recurred when they were restarted, finally resolving completely after she was treated with high-dose pulse steroids for her lupus.
坏死松解性肢端红斑是一种新描述的病症,其特征为手足背部出现边界清晰的鳞屑性斑块。自1996年以来已报告了30多名患者,所有患者均有抗丙型肝炎病毒抗体。一名32岁的台湾女性约10年前被诊断为系统性红斑狼疮伴狼疮性肾炎并接受治疗。此后不久,她注意到足部有几个边界清晰的角化性斑块,边界有红斑,足底未受累。组织病理学显示弥漫性角化不全伴中性粒细胞浸润、颗粒层减少、角质形成细胞上层苍白、散在和成群的角化不良细胞、银屑病样增生以及真皮上层轻度淋巴细胞浸润。经过三次活检后做出诊断。病变在月经前和月经期间经常加重,但孕酮和雌激素的斑贴试验及皮内试验均为阴性。没有乙型肝炎或丙型肝炎感染的证据。病变对锌治疗无反应。停用皮质类固醇后皮疹自行消退,重新使用时复发,最终在她因狼疮接受大剂量脉冲类固醇治疗后完全消退。