Table 1.

Four Types of Pediatric Kaposi Sarcoma

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.