Kaposi’s Sarcoma (KS) is a very peculiar disease, considered as a multifocal vascular neoplasm of reactive origin, associated with HHV8 infection. Differences in presentation and prognosis distinguish 4 well-defined forms: Classic KS, Endemic (African) KS; Tranplant-associated KS, Epidemic or AIDS-associated KS. Classical KS corresponds to the original Kaposi’s description, which predominantly affects elderly males, with a puzzling geographic distribution: otherwise rare, the incidence becomes higher among Jews and people of Mediterranean descent. Presentation is characterised by angiomatous plaques and nodules initially confined to the lower limbs, centripetally spreading all over the body over time, never associated to HIV infection or immunosuppressive treatment. Its evolution is usually slowly, with a very chronic course, completely different from the AIDS associated KS. Treatment is deceiving being the tumour multifocal and the use of chemotherapy affecting patient’s quality of life more than the disease itself. Nevertheless, classic KS may in any time be complicated by a sudden involvement of internal organs as well as onset of complications, and careful monitoring of patient’s condition is essential in the long period. A revision of current literature is presented with the occasion of a ten year case collection to highlight dermatologist engagement, not only in the diagnostic task and the long-term management of the patient, but to challenge improvement of basic research, prevention and treatment, which are still very deceiving points.
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