DOI: 10.21276/sasjs.2018.4.4.1

Pages: 65-68

Xeroderma pigmentosum is a rare photodermatosis of genetic origin, with an autosomal recessive inheritance characterized by increased sensitivity to ultraviolet light. We conducted a retrospective study within the department of plastic, reconstructive, aesthetic surgery and burned of the Mohammed VI University Hospital of Marrakech, over 11 years, which allowed to collect 21 cases of children with xeroderma pigmentosum who have all benefited from at least one surgical intervention in our department. The average age of our patients at the first consultation was 10 years old, with extremes between 3 and 26 years old. A male predominance was noted with 12 boys for 9 girls. The concept of solar exposure was constant in all our patients. Inbreeding of parents was found in 87% of cases (18 patients) and a similar case in fraters was found in 6 patients. On the cutaneous level, all patients had pigmented macules in the photoexposed areas associated with cutaneous xerosis. The preferred site of tumors was the photoexposed areas and more particularly the cephalic extremity in 100% of cases.