TABLE I.

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