PHAKOMATOSIS PIGMENTOVASCULARIS WITH A SELECTIVE IGA DEFICIENCY

We report a 1-year-old boy with an extensive cutaneous vascular malfor mation, oculocutaneous pigmentation, and severe neurologic abnormaliti es from birth, as well as a selective IgA deficiency. Ultrastructural study demonstrated prominent endothelial cells in the lumina of the bl ood vessels. The diagnosis of phacomatosis pigmentovascularis type IIb seemed appropriate for this patient.