Kaposi sarcoma (KS), first described by Moritz Kaposi in 1872, is a multifocal endothelial proliferation of low‐grade malignant potential that is caused by human herpesvirus 8 infection. Debate remains as to whether this is a true neoplasm or a reactive process, although there is evidence of monoclonality, and its precise histogenesis is thought to be lymphatic. A strong predisposition among immunocompromised individuals reflects a dependence upon host immune status, with an over 20‐fold global variation in incidence. There are four distinct clinicopathological subtypes: classic, endemic, iatrogenic and AIDS associated. The most distinguishing clinical features are the rate of progression and the degree of non‐cutaneous involvement. Disease outcome depends heavily upon tumour extent and systemic involvement, and no single approach is definitively curative. KS is highly radiosensitive and immunomodulators are frequently effective, and co‐treatment of HIV infection or reduction of immunosuppression often results in the regression of KS.

Keywords Kaposi sarcoma, human herpesvirus 8, multiple haemorrhagic sarcoma, multiple pigment sarcoma, immune status, acquired immune deficiency syndrome, AIDS, human immunodeficiency virus, HIV