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Phacomatosis Pigmentovascularis with a Selective IgA Deficiency

1995; Wiley; Volume: 12; Issue: 2 Linguagem: Inglês

10.1111/j.1525-1470.1995.tb00145.x

1525-1470

Margarita Larralde de Luna, María Antonia Barquín, José G Casas, Susana Sidelsky,

Vascular Malformations and Hemangiomas

Abstract: We report a 1‐year‐old boy with an extensive cutaneous vascular malformation, oculocutaneous pigmentation, and severe neurologic abnormalities from birth, as well as a selective lgA deficiency. Ultrastructural study demonstrated prominent endothelial cells in the lumina of the blood vessels. The diagnosis of phacomatosis pigmentovascularis type llb seemed appropriate for this patient.