Xeroderma pigmentosum (XP) in the tropics: A case in Guinea with squamous cell carcinoma

B. Diané, M. Bangoura, M. Savané, F. Keita, Mamadou Diouldé Kanté, Mariam Touré, Issiaga Camara, Mohamed Marie Doumbouya, Fanta Kaba, Aissatou Bobo Diallo, Mariama Saliou Tuoré, Mariama Bobo Bah, hierno Mamadou Tounkara, M. Soumah, M. Keita, Mohamed Cissé
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Abstract

XP is generally considered to be a very serious disease in the tropics due to its pronounced sensitivity to sunlight. A disabling condition, first described by Kaposi in 1870, remains very rare in black people. We report a case of squamous cell carcinoma arising from XP, which is rare in dermatological consultations in Guinea. An 11-year-old Guinean student presented with an ulcerative-crust nodule of the scalp that had been evolving for 2 months. He was from a 1st degree consanguineous marriage and had undergone unsuccessful medical and traditional consultations and treatments. Examination showed an ulcerative-crusty nodule on the left parietal area, hypo-hyperpigmented macules on the exposed areas, cheilitis and photophobia. The rest of the examination was normal. Skin histopathology confirmed squamous cell carcinoma, and the CT scan showed no metastatic lesions. Multidisciplinary management was proposed. The patient remains under medical supervision. Skin cancers occurring in XP patients can be prevented by applying photoprotection measures, and genetic counselling before consanguineous unions could also help prevention.
热带地区的色素沉着病(XP):几内亚一例鳞状细胞癌患者
在热带地区,XP 通常被认为是一种非常严重的疾病,因为它对阳光非常敏感。卡波西(Kaposi)于 1870 年首次描述了这种致残性疾病,但它在黑人中仍然非常罕见。我们报告了一例由 XP 引发的鳞状细胞癌病例,这在几内亚的皮肤科就诊病例中非常罕见。一名 11 岁的几内亚学生因头皮溃疡性结节就诊,病程已持续 2 个月。他来自一级近亲结婚,曾接受过医疗和传统咨询及治疗,但均未获成功。检查显示他的左顶叶部位有一个溃疡性结节,暴露部位有色素减退斑、颊炎和畏光。其他检查均正常。皮肤组织病理证实为鳞状细胞癌,CT 扫描显示没有转移病灶。建议进行多学科治疗。患者仍在接受医疗监护。XP患者的皮肤癌可以通过采取光防护措施来预防,近亲结婚前的遗传咨询也有助于预防。
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