manifestations oculaires dans les xeroderma pigmentosum [a propos de 5 cas]

ABSTRACT

Xeroderma pigmentosum is a rare autosomal recessive heredity skin disorder characterised by a defective DNA repair after skin exposure to UV light. Clinical features include skin lesions, with a high incidence of neoplasms, ocular and neurologic manifestations. Ocular signs are severe photophobia, conjunctivitis, corneal scarring, conjunctival noevi, conjunctival and eyelids neoplasms. A retrospective study of 5 cases of xeroderma pigmentosum including children aged 4 to 11 years old were carried out complications which included squamous cell carcinoma and ectropion by lower eyelid retraction were treated surgically. The authors present the ocular manifestations in xeroderma pigmentosum, discuss their treatment and prognosis which depends mainly on recurretlces