Epidermolysis bullosa (EB) defines a group of rare, inherited dermatoses that present with repeated blistering, erosions, and ulceration in response to seemingly disproportionate mechanical trauma. This occurs due to genetically-mediated skin fragility defects within the dermo-epidermal junction. An extensive phenotypic range is described. Some forms of epidermolysis bullosa place patients at an increased risk of infection, disabling deformities secondary to heavy scarring, and aggressive cutaneous malignancy, often causing early fatality. A broad range of extra-cutaneous manifestations can lead to significant morbidity and premature mortality. In the absence of curative treatment, the current focus of care prioritizes preventing and relieving symptoms and their complications. More recently, novel therapeutics, including topical gene therapies, have gained approval for increased symptomatic improvement of this otherwise intractable group of conditions. This activity outlines the presentation, pathophysiology, evaluation, and management of epidermolysis bullosa, and describes the vital role of the interdisciplinary team in treating extra-cutaneous complications. Areas of research are highlighted pertaining to preventative and abortive clinical strategies, emphasizing the use of gene replacement therapy.
- Provider:StatPearls, LLC
- Activity Link: https://www.statpearls.com/ArticleLibrary/viewarticle/158627
- Start Date: 2024-12-01 06:00:00
- End Date: 2024-12-01 06:00:00
- Credit Details: AMA PRA Category 1 Credit™️: 1.0 hours
Nursing: 1.0 hours
Pharmacy: 1.0 hours - MOC Credit Details: ABS - 1.0 Point; Credit Type(s): Accredited CME (ABS)
ABOS - 1.0 Point; Credit Type(s): Accredited CME (ABOS)
ABPATH - 1.0 Point; Credit Type(s): Lifelong Learning (ABPATH)
ABA - 1.0 Point; Credit Type(s): Lifelong Learning (ABA)
ABTS - 1.0 Point; Credit Type(s): Accredited CME (ABTS)
ABIM - 1.0 Point; Credit Type(s): Medical Knowledge (ABIM)
ABS - 1.0 Point; Credit Type(s): Self-Assessment (ABS)
ABP - 1.0 Point; Credit Type(s): Lifelong Learning and Self-Assessment (ABP)
ABOHNS - 1.0 Point; Credit Type(s): Self-Assessment (ABOHNS)
ABTS - 1.0 Point; Credit Type(s): Self-Assessment (ABTS) - Commercial Support: No
- Activity Type: Enduring Material
- CME Finder Type: Online Learning
- Fee to Participate: Variable
- Measured Outcome: Learner Knowledge, Learner/Team Competence
- Provider Ship: Directly Provided
- Registration: Open to all
- Specialty: Adolescent Medicine, Cardiovascular Disease, Dermatopathology, Endocrinology, Diabetes, and Metabolism, Gastroenterology, General Otolaryngology, General Pediatrics, General Surgery, General Thoracic, Hematology, Hospital Medicine, Internal Medicine, Laryngology, Molecular Genetic Pathology, Neonatal-Perinatal Medicine, Nephrology, Non-Thoracic Surgery, Pain Medicine, Pediatric Cardiology, Pediatric Critical Care Medicine, Pediatric Emergency Medicine, Pediatric Endocrinology, Pediatric Gastroenterology, Pediatric Hematology-Oncology, Pediatric Nephrology, Pediatric Pulmonology, Pulmonary Disease, Rheumatology, Surgery of the Hand