Xeroderma pigmentosum : about twelve Mauritanian cases.

Abstract

In order to describe the epidemiological and clinical features of xeroderma pigmentosum (XP) in Mauritania, we conducted a retrospective study of XP cases encountered in the dermatology department of the National Center Hospital of Nouakchott in Mauritania. From January 2012 to March 2016, all patients with XP records were reviewed. XP diagnosis was based on clinical evidences (xerosis, lentigo, poikiloderma and photophobia) and in some cases it was confirmed by genetic studies. We analyzed the following data: age, sex, clinical signs, presence of tumor. In total, 12 cases of XP were identified: 8 male and 4 female, so the sex ratio was 2. The age of patients ranged from 2 to 13 years with an average of 7 years. Clinically, xerosis was found in all patients, poïkilodermia was noted in 11 patients. Skin cancers encountered were: basal cell carcinoma 3 cases, squamous cell carcinoma 1 case. XP is a rare but serious pathology in Mauritania because of many obstacles in preventing complications of this disease, such as permanent sunshine and ignorance of people.