XERODERMA PIGMENTOSUM (Kaposi) Under the name of Xeroderma pigmentosum, Kaposi described in 1870 a chronic affection of the uncovered parts of the skin occurring under the influence of the rays of the sun, which leads to pigmentation and atrophy and is accompanied by the formation of malignant tumors.
Clinical brownish mactike resembling freales, appear on the skin, sometimes in the first year of life, which make the skin look spotted. In the interstices there are dilatation of the vessels, small verrucous formations, papillary and scaly deposits. The skin between thcse spots looks white and atrophic. Gradually the affection spreads, the pigmentation of the skin increases, and the skin becomes more and more atrophic. There xerosis of the conjunctiva, eetropium, ulcers and fissures of the skin, and multiple pigmented sarcomata and carcinomata may appear even in the third year.
The affection occurs in earliest life (first and second year) and is doubtless attributable to a family disposition. Although parents and children are never affected simultaneously and although heredity can never be demonstrated, the influence of race and family disposition is unquestionable. According to Halle the disease .seized Sti families in 1S6 cases, Forster found 150 positive cases distributed among 96 fami lies. Besides there was consanguinity of 11.5 per cent. as against the average frequency of 6 to 11 per cent. in marriages of relatives. It is
remarkable that usually the children of the samc sex are affected in a family. Jewish children seem to be specially prone to this affection, Eisenberg observing 24 per cent. of Jews in 52 cases. The affection is in all probability, caused by a hypersensitiveness to light, as it usually occurs at the uncovered parts of the body, the face, hands and, if exposed, the feet.
Pathology.—Microscopic examination of the first stage shows an accumulation of round cells in the papillary body and stratum sub papillare (Lucasiewitz), and a distinct cedema around the blood vessels and glands. In the later stages there is atrophy of the tissue in the white atrophic places, while in the hyper-pigmented places there is a thickening of the papillary body, and in it cellular infiltrates together with considerable accumulation of pigment. The vessels, especially the capillaries, are considerably changed.
Etiology.—As to the cause of the affection itself and the develop ment of sarcoma and carcinoma, we are as yet completely in the (lark.
The diagnosis does not offer any difficulties.
Treatment.—The treatment is powerless to remove the cause; perhaps it is possible to eliminate the influence of the light by protect ing the skin through colored veils, etc., perhaps it is also possible to effect a cure. of the carcinoma and sarcoma by phototherapy.