Congenital Ichthyosis
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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
1Division of Neonatology and Developmental Biology, Department of Pediatrics, David Geffen School of Medicine, University of California-Los Angeles, Los Angeles, California.
Abstract:
See bonus NeoBriefs videos and downloadable teaching slides on this article's homepage Congenital ichthyosis (CI) encompasses a heterogenous group of skin disorders characterized by abnormal epidermal differentiation. Many present in the newborn period, and affected patients are at risk of serious complications. A compromised skin barrier places these infants at risk for transepidermal water loss, temperature dysregulation, compromised limb perfusion, and sepsis. It is important for neonatal clinicians to identify these disorders at birth and to create individualized management plans. This review seeks to summarize the signs of CI in the neonatal period and common management strategies.
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