[Kaposi's sarcoma--classic form. A rare familial case]

Abstract

Although Kaposi's sarcoma became very well-known with the outbreak of AIDS, this pathology in the classic Mediterranean form is still unusual and obscure. Nowadays we identify four types of Kaposi's sarcoma: 1) the classic Mediterranean form, 2) the endemic African one, 3) the therapeutical in immunosuppressed patients, 4) the epidemic AIDS-related one. We report a very rare case of familial Kaposi's sarcoma: the father (72 y.o.) manifested Kaposi's sarcoma in 1989 with several angiomatoid nodules on the penis and hands; the son (31 y.o.) presented in 1991 only one little nodule on the penis. The right therapy in these cases (patients with a small number of localizations) was to remove all the tumors and to wait: whereas in other cases it's preferable to use radio- or chemo-therapy or immunomodulator agents. We studied the patients and identified their form of Kaposi like the classic one not completely related (the son) to the HLA DR5, which is the HLA-phenotype most frequent in this sarcoma. Moreover we stress the rarity of genital localization in the classic form, instead it is very common (20%) in the AIDS-related one.