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Junctional Epidermolysis Bullosa Incidence and Survival: 5-Year Experience of the Dystrophic Epidermolysis Bullosa Research Association of America (DebRA) Nurse Educator, 2007 to 2011

Authors

  • Geraldine Kelly-Mancuso R.N.,

    Corresponding author
    1. Dystrophic Epidermolysis Bullosa Research Association of America, New York, New York
    • Address correspondence to Geraldine Kelly-Mancuso, R.N., Dystrophic Epidermolysis Bullosa Research Association of America, 16 East 41st Street, 3rd Floor, New York, NY 10017, or e-mail: nursegeri@debra.org.

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  • Brett Kopelan M.A.,

    1. Dystrophic Epidermolysis Bullosa Research Association of America, New York, New York
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  • Richard G. Azizkhan M.D.,

    1. Cincinnati Children's Epidermolysis Bullosa Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
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  • Anne W. Lucky M.D.

    1. Cincinnati Children's Epidermolysis Bullosa Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
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Abstract

Junctional epidermolysis bullosa (JEB) is a particularly devastating type of epidermolysis bullosa, especially in the newborn period. Data about the number of new cases of JEB in the United States were collected from the records of the Dystrophic Epidermolysis Bullosa Research Association of America (DebRA) nurse educator. Seventy-one children with JEB were reported to have been born in the 5 years between 2007 and 2011, reflecting an incidence of at least 3.59 per million per year, significantly higher than previously estimated (2.04 per million). There was a high prevalence of morbidity and infant mortality of at least 73%, as 52 of the 71 cases proved fatal by June 2012. These data emphasize the need for future research to develop treatment and ultimately a cure for this disorder.

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