Background: Kaposi sarcoma (KS) is a cutaneous endothelial vascular proliferation with four subtypes: iatrogenic, acquired immune deficiency syndrome (AIDS) related, African, and classic. Familial cases of KS are rare, with 72 cases reported to date, and all were described with the classic variant. The occurrence of classic KS in the Jewish population is well documented, and most of the familial classic KS cases were also reported in Jewish families.
Objective: We briefly present the history, biopsies, laboratory data, diagnosis, and treatment of localized lower limb classic KS in two siblings of Jewish Eastern European ethnic descent with their response to different therapy modalities. One of our cases had the second longest reported period of follow-up for familial classic KS of 40 years.