LETTERS TO THE EDITOR Xeroderma pigmentosum in three consecutive siblings of a Nigerian family: observations on oculocutaneous manifestations in black African children

EDITOR,—Xeroderma pigmentosum (XP), a rare autosomal recessive disorder characterised by defective DNA repair leading to clinical and cellular hypersensitivity to ultraviolet radiation, manifesting mainly as intolerance of skin and eyes to light, has been described in all races, but is exceedingly rare in the negroid race, although some cases have been reported in both the American and African black people. We describe three consecutive siblings of a Nigerian, Fulani, family with the typical features of XP. We wish to draw attention to the clinical, phenotypic variations of this syndrome in black children of the same family living together in an area of high sunshine, and the diYculties in the management of XP patients with advanced disease and limited access to facilities in an environment where avoidance of skin exposure to intense ultraviolet rays is problematic. We believe the patients we have described constitute the first series on XP in black children in the west African subregion.