Malipatil Sharanabasappa Rudragouda, Jonnalagadda Sriharsha Vijay
Shri B.M Patil Medical College & Research Centre, Vijayapura, India.
Indian J Otolaryngol Head Neck Surg. 2019 Oct;71(Suppl 1):702-705. doi: 10.1007/s12070-018-1508-z. Epub 2018 Oct 3.
Xeroderma pigmentosa (XP) is a rare autosomal recessive disorder which is characterized by a defect in nucleotide excision repair of DNA following exposure to UV radiation. This leads to hypersensitivity to sunlight causing pigmented skin lesions, photophobia and a 1000-fold increase in risk of developing cutaneous malignancies like basal cell carcinomas, squamous cell carcinomas and melanomas of head and neck. We present an interesting case of a 28 year old man with basosquamous cell carcinoma secondary to XP treated successfully with surgical excision and reconstruction with rotational flap technique.
着色性干皮病(XP)是一种罕见的常染色体隐性疾病,其特征是暴露于紫外线辐射后DNA核苷酸切除修复存在缺陷。这导致对阳光过敏,引起皮肤色素沉着性病变、畏光,以及患皮肤恶性肿瘤(如基底细胞癌、鳞状细胞癌和头颈部黑色素瘤)的风险增加1000倍。我们报告了一例有趣的病例,一名28岁男性因着色性干皮病继发基底鳞状细胞癌,通过手术切除并用旋转皮瓣技术重建而成功治愈。