Table 1.
Type of KS | Relative frequency | Geographic characteristic | Immunodeficiency | Clinical presentation |
---|---|---|---|---|
Epidemic | Relatively common | Worldwide | AIDS | Aggressive; sometimes without cutaneous involvement, oftentimes involving mucosa and visceral organs, IRIS |
Endemic | Rare | Sub‐Saharan Africa | Not yet deciphered | Generalized or localized lymphadenopathy with sparse mucosal or skin lesions, if any |
Iatrogenic | Very rare | Developed world | Immunosuppressive therapy, (e.g. transplantation) | Variable; lymphadenopathy, visceral, mucocutaneous or cutaneous involvement |
Classic | Exceedingly rare | Mediterranean Basin, Eastern European | WAS, IFN‐γR1 deficiency, STIM1 deficiency, OX40 deficiency | Rapidly progressive disseminated and aggressive cutaneous lesions, oftentimes with mucosal and lymph node involvement |
Abbreviations: KS, Kaposi sarcoma; AIDS, acquired immunodeficiency syndrome; IRIS, immune reconstitution inflammatory syndrome; WAS, Wiskott–Aldrich syndrome.