[Klippel-Trenaunay syndrome]

The author in connection with three properly observed female patients summarizes the clinical entity of Klippel-Trenaunay syndrome, its partly resembling partly dissociating features facing to other vascular congenital anomalies, and the most important aspects of vasculogenesis. Based on the observation, attention is distinctly called on the first case in whom the Klippel-Trenaunay syndrome presenting with multiple mesenchymal anomalies was associated with selective congenital IgA deficiency. The second was the daughter of this first patient, who also had the syndrome and a selective congenital IgA deficiency. The other daughter of the patient was free from both of the pathological conditions. The third case represented the features of the typical syndrome without verified immunodeficiency. The literary data do not speak about possible correlations between Klippel-Trenaunay syndrome and immunodeficiencies. The pathogenesis of the process and of its possible connections with immunodeficiencies remain to be investigated.