Much confusion surrounds a rare and occasionally described variant of Kapos
i's sarcoma (KS) known as lymphangioma-like or bullous KS. We describe the
typical clinical and histologic features and elucidate the etiologic cell i
n lymphangioma-like KS. A computer-based review of the English-language lit
erature was performed. Routine histologic and immunoperoxidase techniques w
ere performed on formalin-fixed tissue. Immunoperoxidase staining for anti-
CD31, anti-CD34, and anti-factor VIII-related antigen suggests that the eti
ologic cell of origin is the vascular endothelial cell. Lymphangioma-like K
S has been described for more than a century and given various names. Dilat
ed vascular spaces correlate with the clinically bullous lesions, which are
veritable KS and not a secondary reaction to it.