[Current aspects of xeroderma pigmentosum: apropos of two new cases]. / Aspects actuels du "Xeroderma pigmentosum": à propos de deux nouvelles observations.

Two recent cases of children treated for xeroderma pigmentosum are reported, and knowledge of the disease updated based on numerous reports published over the last few years, particularly by french authors ( Vaillant et al., 1979 and Mouly , Dufourmentel and Papadopoulos , 1980). Points discussed include the similarity of histology and course of the malignant cutaneous lesions with those of adult outdoor workers (co- carcinogenic role of ultraviolet rays ?), and the effect of the introduction of vitamin A acid into treatment on the course of the general and cutaneous lesions (immunocompetence, tumoral growth rate). A further feature discussed is the question raised by the surgical attitude: radical, extensive and mutilating as in the first case, or limited and conservative, with consequent long-term inconveniences, as in the second case. The persistence of an extremely poor prognosis after more than a century of study of the disease (it received its name from Kaposi as early as 1882. . . !!) is a strong argument for imposing the preventive measures which are still applied with difficulty at the present time.