Epidermolysis Bullosa News

    Skip to content
    • Home
    • What Is EB?
      • Types of EB
        • Dystrophic Epidermolysis Bullosa
        • Epidermolysis Bullosa Acquisita (EBA)
        • Epidermolysis Bullosa Simplex
        • Junctional Epidermolysis Bullosa
        • Kindler Syndrome
      • Causes of EB
      • Epidermolysis Bullosa Inheritance
      • Symptoms of EB
      • Diagnosis of EB
      • Prognosis & Life Expectancy
      • Living with EB
    • Treatment
      • Filsuvez
      • Experimental Treatments
        • B-VEC (KB103)
        • CCP-020 Cream
        • EB-101 for RDEB
        • FCX-007
        • SD-101 Cream for Epidermolysis Bullosa
    • Columns
      • Fragile but Fierce
      • Girl with the Butterfly Tattoo
    • COVID-19 Info
      • COVID-19 Updates

    Prognosis Good for Pediatric Autoimmune Blistering Diseases, but Long-Term Follow-up Needed, Study Says

    March 4, 2019
    Alejandra Viviescas, PhDby Alejandra Viviescas, PhD

    In News.

    Prognosis Good for Pediatric Autoimmune Blistering Diseases, but Long-Term Follow-up Needed, Study Says

    Click here to subscribe to the Epidermolysis Bullosa News Newsletter!

    0
    (0)

    Autoimmune blistering diseases, including epidermolysis bullosa, in children have a better prognosis than in adults, but long-term follow-up is necessary for adequate management due to frequent late relapses, a study suggests.

    The study, “Long-term evolving profile of childhood autoimmune blistering diseases. Retrospective study on 38 children,” was published in the Journal of the European Academy of Dermatology and Venereology.

    Autoimmune blistering diseases are a group of disorders in which the immune cells of the body attack the different layers of the skin causing blisters. The conditions receive different names depending on which part of the skin is attacked.

    These conditions usually appear during adulthood, but there are some cases in children. Little is known about the evolution and occurrence of pediatric autoimmune blistering diseases.

    Researchers at the Necker-Enfants Malades Hospital in Paris characterized the long-term evolution of 38 individuals who developed autoimmune blistering diseases before the age of 16. They started treatment at 4.9 years old on average and were treated between January 1993 and December 2015.

    A total of 18 participants had linear immunoglobulin A disease, 10 had pemphigus, five had herpetiform dermatitis, four had bullous pemphigoid, and one had epidermolysis bullosa aquisita (EBA).

    The researchers collected clinical data from the hospital database and obtained long-term data by performing a phone survey.

    Patients received treatment for an average of 30.6 months. All patients were followed for at least one year, with an average follow-up of 6.6 years.

    During the follow-up period, 29 patients had at least one relapse, including eight late relapses, defined as those occurring more than six months after initial healing. Most relapses occurred about three months after initial healing.

    Treatment with corticosteroids applied to the skin led to a complete remission in seven patients (three with linear immunoglobulin A disease, three with bullous pemphigoid, and one with pemphigus).

    A combination of topical corticosteroids and other medicines such as oral corticosteroids, dapsone, mycophenolate mofetil or Rituxan (rituximab) put another 19 patients in complete remission.

    A total of 34 children (89%) achieved complete remission, 26 of whom were out of treatment by the last follow-up.

    The outcome in children was better than in adults, where only a third of all cases achieve complete remission.

    “Childhood [autoimmune blistering diseases] appear to be of good overall prognosis but a long-term follow-up is mandatory, as relapses can be late. The use of topical corticosteroids is frequently effective alone or in association,” the researchers wrote.

    Two cases in the study were associated with underlying primary immune deficiency — a condition that weakens the immune system, making the individuals more susceptible to infections and diseases.

    “In the pediatric population, and particularly when manifestations of auto-immunity occur very early in the life, the question of an underlying primitive [abnormal immune system] has to be considered,” the investigators said.

    They also suggested that topical corticosteroids, rather than oral corticosteroids, should be the first line of treatment for pediatric autoimmune blistering diseases, although some conditions such as EBA and pemphigus often require oral treatment.

    • Author Details
    Alejandra Viviescas, PhD
    Alejandra has a PhD in Genetics from São Paulo State University (UNESP) and is currently working as a scientific writer, editor, and translator. As a writer for BioNews, she is fulfilling her passion for making scientific data easily available and understandable to the general public. Aside from her work with BioNews, she also works as a language editor for non-English speaking authors and is an author of science books for kids.
    ×
    Alejandra Viviescas, PhD
    Alejandra has a PhD in Genetics from São Paulo State University (UNESP) and is currently working as a scientific writer, editor, and translator. As a writer for BioNews, she is fulfilling her passion for making scientific data easily available and understandable to the general public. Aside from her work with BioNews, she also works as a language editor for non-English speaking authors and is an author of science books for kids.
    Latest Posts
    • wound care and topical anesthetic
    • granzyme B, EBA
    • survey, clinical trials
    Print This Article

    How useful was this post?

    Click on a star to rate it!

    Average rating 0 / 5. Vote count: 0

    No votes so far! Be the first to rate this post.

    As you found this post useful...

    Follow us on social media!

    We are sorry that this post was not useful for you!

    Let us improve this post!

    Tell us how we can improve this post?

    Tagged Autoimmune blistering diseases, children, long-term follow up, prognosis, remission.

    Post navigation

    Previous: WODC 2019 Organizers Expect 1,200 to Attend Rare Disease Conference in April
    Next:NIH Rare Disease Day Highlights Joint Networks Advancing Array of Research

    Recent Posts

    • When EB Steals Your Cat

      November 19, 2020

    • Latest NORD Webinar Offers Insights on Starting Nonprofit, Patient Registry

      November 19, 2020

    • Venture Into Cures event

      Eddie Vedder Hosts ‘Venture Into Cures,’ Virtual Event Supporting EB Research

      November 18, 2020

    Featured

    National Survey Seeks to Assess Full Social, Economic Burdens of Rare Diseases in US

    July 9, 2020

    rare disease survey

    Alexion Charitable Foundation Giving $1.1M to Assist Rare Disease Community During Pandemic

    June 22, 2020

    PTR-01 Shows Signs of Efficacy in RDEB Patients, Early Trial Data Reveal

    May 27, 2020

    Filsuvez trial

    Create your own user feedback survey

    Epidermolysis Bullosa News

    BioNews Services, LLC
    3 W Garden St
    Suite 700
    Pensacola, FL 32502
    Email: [email protected]
    Phone: +1-800-936-1363
    • Publishing Team
    • Leadership
    • Our Values
    • Corrections Policy
    • Contact Us
    • Terms of Service
    • Privacy Policy
    • Disable Notifications

    Disclaimer:

    Epidermolysis Bullosa News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

    Copyright © 2013-2020 All rights reserved.
      We use cookies to enhance your experience on our website. Privacy Policy Accept