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Updated: Aug 7, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
Phacomatosis pigmentovascularis with a selective IgA deficiency
M L de Luna1, M A Barquin, J G Casas
1Department of Pediatric Dermatology, Buenos Aires University, Hospital General de Agudos Jose Maria Ramos Mejía, Argentina.
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 IgA deficiency. Ultrastructural study demonstrated prominent endothelial cells in the luminal of the blood vessels. The diagnosis of phacomatosis pigmentovascularis type IIb seemed appropriate for this patient.
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