Onishi Masazumi, Tsunoda Kanako, Maeda Fumihiko, Moriwaki Shinichi, Amano Hiroo
Department of Dermatology, Iwate Medical University School of Medicine, Yahaba-cho, Japan.
Department of Dermatology, Osaka Medical College, Takatsuki, Japan.
Case Rep Dermatol. 2020 Aug 18;12(2):144-149. doi: 10.1159/000508884. eCollection 2020 May-Aug.
Xeroderma pigmentosum (XP) is an inherited autosomal recessive disorder characterized by photosensitivity and an increased risk of developing multiple skin neoplasms at sites exposed to the sun. We report a 73-year-old Japanese man with angiosarcoma of the auricle and an XP-variant, which is a very rare condition. In this case, long-term physical stimulation due to auricular deformation after surgery may have been the cause. Angiosarcoma associated with XP has a better prognosis than common angiosarcoma, perhaps because of the smaller tumor size. As XP patients are at high risk of skin neoplasms, they consult dermatologists regularly, and therefore skin tumors are likely to be detected early.
着色性干皮病（XP）是一种常染色体隐性遗传性疾病，其特征为光敏感性以及在暴露于阳光的部位发生多种皮肤肿瘤的风险增加。我们报告了一名73岁的日本男性，患有耳廓血管肉瘤及一种非常罕见的XP变异型。在该病例中，手术后耳廓变形导致的长期物理刺激可能是病因。与XP相关的血管肉瘤比普通血管肉瘤预后更好，这可能是因为肿瘤体积较小。由于XP患者患皮肤肿瘤的风险较高，他们会定期咨询皮肤科医生，因此皮肤肿瘤很可能被早期发现。
