touchDERMATOLOGY touchDERMATOLOGY
Start activity
 
What are key characteristics of the more severe forms of EB, namely junctional EB (JEB) and dystrophic EB (DEB)?
What is the underlying pathophysiology causing skin fragility in patients with JEB and DEB?
What issues do patients with EB have that further complicate their ability to heal?
What are the extracutaneous manifestations that have a negative impact on QoL in EB? What are the psychosocial impacts on QoL?
What is the rationale and evidence for newly available and emerging topical treatments for patients with EB?
Can you give an overview of who should be involved in the multidisciplinary team supporting patients with EB?
How can topical and grafting therapies best be integrated in a supportive MDT care approach in clinical practice?
Take CE/CME Test
Dermatological Disorders, Paediatric Dermatology, Rare Diseases CE/CME accredited

touchFOCUS
Quickfire Q&A with expert faculty. Close

Supporting healing in epidermolysis bullosa: Innovations and new directions for care

Take CE/CME Test

Prof. Amy Paller, MS, MD is the Walter J. Hamlin professor and chair of dermatology, professor of paediatrics, and principal investigator of the NIH-funded Skin Biology and Diseases Resource-based Center (SBDRC) at Northwestern University Feinberg School of Medicine in Chicago, USA. She served as chief of paediatric dermatology at Northwestern’s Children’s Hospital until she became chair of dermatology at Northwestern in 2004. read more

Prof. Paller is an NIH-funded investigator in both her laboratory-based and clinical research. She has directed the Pediatric Dermatology Clinical Trials Unit at Northwestern/Lurie Children’s Hospital for the past 30 years, and has been the lead investigator on several landmark trials in paediatric skin disease, particularly related to atopic dermatitis, psoriasis and genodermatoses. Her laboratory focuses on wound healing, skin innervation in inflammatory diseases, and topically delivered gene regulation through nanotherapy.

Prof. Paller was the inaugural president of the International Eczema Council and inaugural co-president of the Pediatric Dermatology Research Alliance. She has been president of the Society for Investigative Dermatology, Society for Pediatric Dermatology, and the Women’s Dermatologic Society. She is currently president of the International Society for Pediatric Dermatology and president-elect of the American Dermatological Association.

An author of almost 700 peer-reviewed publications, Prof. Paller has edited several major textbooks in dermatology, among them Hurwitz’s Clinical Pediatric Dermatology and Fitzpatrick’s Dermatology in General Medicine. She has received several honours for her scholarship, leadership and mentorship, among them election to the American Association of Physicians and the American Dermatological Association, the Founder’s Award from the Chicago Dermatological Society, the International Leadership Award from the International League of Dermatological Societies, and the inaugural Hall of Fame Award from the Society for Pediatric Dermatology.

Prof. Amy Paller discloses: Investigator fees from AbbVie, Applied Pharma Research, BioMendics, Dermavant, Eli Lilly, Incyte, Janssen, Krystal, Regeneron, Timber and UCB. Consultancy fees from Abeona, Apogee, Arcutis, Aslan, BioCryst, Boehringer Ingelheim, Bristol Myers Squibb, Castle Creek, Dermavant, Incyte, Krystal, LEO Pharma, L’Oreal, Mitsubishi Tanabe, MoonLake Immunotherapeutics, Procter and Gamble, Regeneron, Sanofi, Synergy and UCB. Data safety monitoring board fees from AbbVie, Abeona and Galderma.

Learning Objectives

After watching this activity, participants should be better able to:

  • Outline the key features of EB, including its pathophysiology and clinical manifestations
  • Evaluate the role of new and emerging topical therapies in the multidisciplinary care of patients with EB
Overview

In this interview, Prof. Amy Paller answers questions on the management of patients with epidermolysis bullosa. The interview will centre on the key features of EB, including its pathophysiology and clinical manifestations, and the role of new and emerging topical therapies in patient care, with a focus on a multidisciplinary team approach.

This activity is funded by an independent medical education grant from Chiesi USA Inc. This activity is jointly provided by USF Health and touchIME. read more

Target Audience

Dermatologists, paediatric dermatologists, paediatricians and primary care physicians involved in the management of epidermolysis bullosa.

USF Accreditation

Disclosures

USF Health adheres to the Standards for Integrity and Independence in Accredited Continuing Education. All individuals in a position to influence content have disclosed to USF Health any financial relationship with an ineligible organization. USF Health has reviewed and mitigated all relevant financial relationships related to the content of the activity. The relevant relationships are listed below. All individuals not listed have no relevant financial relationships.

Faculty

Prof. Amy Paller discloses: Investigator fees from AbbVie, Applied Pharma Research, BioMendics, Dermavant, Eli Lilly, Incyte, Janssen, Krystal, Regeneron, Timber and UCB. Consultancy fees from Abeona, Apogee, Arcutis, Aslan, BioCryst, Boehringer Ingelheim, Bristol Myers Squibb, Castle Creek, Dermavant, Incyte, Krystal, LEO Pharma, L’Oreal, Mitsubishi Tanabe, MoonLake Immunotherapeutics, Procter and Gamble, Regeneron, Sanofi, Synergy and UCB. Data safety monitoring board fees from AbbVie, Abeona and Galderma.

Content reviewer

Danielle Walker, DNP, APRN, AGNP-C, has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Contributors

Sola Neunie has no financial interests/relationships or affiliations in relation to this activity.

USF Health Office of Continuing Professional Development and touchIME staff have no financial interests/relationships or affiliations in relation to this activity.

Requirements for Successful Completion

In order to receive credit for this activity, participants must review the content and complete the post-test and evaluation form. Statements of credit are awarded upon successful completion of the post-test and evaluation form.

If you have questions regarding credit please contact [email protected] 

Accreditations

Physicians

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through a joint providership of USF Health and touchIME. USF Health is accredited by the ACCME to provide continuing medical education for physicians.

USF Health designates this enduring material for a maximum of 0.5 AMA PRA Category 1 CreditTM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Advanced Practice Providers

Physician Assistants may claim a maximum of 0.5 Category 1 credits for completing this activity. NCCPA accepts AMA PRA Category 1 CreditTM from organizations accredited by ACCME or a recognized state medical society.

The AANPCP accepts certificates of participation for educational activities approved for AMA PRA Category 1 CreditTM by ACCME-accredited providers. APRNs who participate will receive a certificate of completion commensurate with the extent of their participation.

Date of original release: 27 November 2024. Date credits expire: 27 November 2025.

If you have any questions regarding credit, please contact [email protected]

 

This activity is CE/CME accredited

To obtain the CE/CME credit(s) from this activity, please complete this post-activity test.

Claim Credit

Topics covered in this activity

Dermatological Disorders / Paediatric Dermatology / Rare Diseases
REGISTER NOW FOR FREE ACCESS TO
  • 1000+ topical and insightful peer-reviewed journal articles
  • 100+ hours of bite-sized congress highlights
  • 10 major therapy areas packed with the latest scientific advances
  • 150+ specialties offering learn-on-the-go medical education
  • + Concise email updates and newsletters so you never miss out
Register For Free Now
Claim Credit
touchFOCUS
Supporting healing in epidermolysis bullosa: Innovations and new directions for care
0.5 CE/CME credit

Question 1/3
When talking to the parents of a newly diagnosed infant with dystrophic epidermolysis bullosa regarding the causes of the condition, how would you describe it? “Dystrophic epidermolysis bullosa arises due to…”

Dystrophic epidermolysis bullosa is one of the four recognized clinical subtypes of epidermolysis bullosa. It may be inherited in autosomal dominant or recessive patterns, with the latter giving rise to more severe phenotypes.1,2 Dystrophic epidermolysis bullosa features fragility below the most superficial portion of the dermis called the lamina densa, due to a deficiency in collagen VII protein.1,2 While all four types of epidermolysis bullosa cause repeated blistering, dystrophic epidermolysis bullosa features a defect much deeper in the dermo-epidermal junction, propagating profound mucocutaneous scarring and fibrosis.2

References

  1. DEBRA of America. Available at: https://debra.org/about-eb/eb-depth (accessed 11 November 2024).
  2. Khanna D, Bardhan A. Epidermolysis Bullosa. 2024. In StatPearls. Treasure Island (FL): StatPearls. Available at: www.ncbi.nlm.nih.gov/books/NBK599531/ (accessed 11 November 2024).
Question 2/3
Your patient is a 7-month-old infant who has recently been diagnosed with junctional epidermolysis bullosa and has extensive wounds on the face and the tips of their fingers. Which topical therapy would you recommend to their parents to best assist with wound healing in this patient?

Birch triterpenes is a topical gel indicated for the treatment of wounds associated with dystrophic epidermolysis bullosa and junctional epidermolysis bullosa in adult and paediatric patients 6 months of age and older.1 Beremagene geperpavec is a topical HSV-1–based gene therapy designed to restore C7 protein and is indicated for the treatment of wounds in patients 6 months of age and older with dystrophic epidermolysis bullosa.2,3 Prademagene zamikeracel (pz-cel or EB-101) is an investigational treatment consisting of COL7A1 gene-corrected epidermal sheets for the treatment of recessive dystrophic epidermolysis bullosa.4

Abbreviation

HSV, herpes simplex virus.

References

  1. Birch triterpenes PI. 2023. Available at: www.accessdata.fda.gov/drugsatfda_docs/label/2023/215064s000lbl.pdf (accessed 12 November 2024).
  2. Guide SV, et al. N Engl J Med.2022;387:2211–9.
  3. Beremagene geperpavec-svdt PI. 2023. Available at: https://www.fda.gov/media/168350/download (accessed 12 November 2024).
  4. Tang JY, et al. Presented at: The International Societies for Investigative Dermatology Meeting, 10–13 May 2023, Tokyo, Japan.
Question 3/3
You have recommended the topical HSV-1–based gene therapy, B-VEC, to be used for your infant patient with dystrophic epidermolysis bullosa. The patient's parents ask what outcomes could be expected with this treatment. How would you advise them, based on phase III clinical trial data for this agent?

B-VEC, beremagene geperpavec; HSV, herpes simplex virus.

In a phase III, double-blind, intrapatient, randomized, placebo-controlled trial involving 31 patients aged 6 months or older with genetically confirmed dystrophic epidermolysis bullosa, primary wound pairs were exposed to B-VEC and placebo. At 6 months, complete wound healing occurred in 67% of the wounds exposed to B-VEC as compared with 22% of those exposed to placebo (p=0.002).

Abbreviation

B-VEC, beremagene geperpavec.

Reference

Guide SV, et al. N Engl J Med. 2022;387:2211–9.

Back to Activity
Copied to clipboard!
accredited arrow-down-editablearrow-downarrow_leftarrow-right-bluearrow-right-dark-bluearrow-right-greenarrow-right-greyarrow-right-orangearrow-right-whitearrow-right-bluearrow-up-orangeavatarcalendarchevron-down consultant-pathologist-nurseconsultant-pathologistcrosscrossdownloademailexclaimationfeedbackfiltergraph-arrowinterviewslinkmdt_iconmenumore_dots nurse-consultantpadlock patient-advocate-pathologistpatient-consultantpatientperson pharmacist-nurseplay_buttonplay-colour-tmcplay-colourAsset 1podcastprinter scenerysearch share single-doctor social_facebooksocial_googleplussocial_instagramsocial_linkedin_altsocial_linkedin_altsocial_pinterestlogo-twitter-glyph-32social_youtubeshape-star (1)tick-bluetick-orangetick-red tick-whiteticktimetranscriptup-arrowwebinar Sponsored Department Location NEW TMM Corporate Services Icons-07NEW TMM Corporate Services Icons-08NEW TMM Corporate Services Icons-09NEW TMM Corporate Services Icons-10NEW TMM Corporate Services Icons-11NEW TMM Corporate Services Icons-12Salary £ TMM-Corp-Site-Icons-01TMM-Corp-Site-Icons-02TMM-Corp-Site-Icons-03TMM-Corp-Site-Icons-04TMM-Corp-Site-Icons-05TMM-Corp-Site-Icons-06TMM-Corp-Site-Icons-07TMM-Corp-Site-Icons-08TMM-Corp-Site-Icons-09TMM-Corp-Site-Icons-10TMM-Corp-Site-Icons-11TMM-Corp-Site-Icons-12TMM-Corp-Site-Icons-13TMM-Corp-Site-Icons-14TMM-Corp-Site-Icons-15TMM-Corp-Site-Icons-16TMM-Corp-Site-Icons-17TMM-Corp-Site-Icons-18TMM-Corp-Site-Icons-19TMM-Corp-Site-Icons-20TMM-Corp-Site-Icons-21TMM-Corp-Site-Icons-22TMM-Corp-Site-Icons-23TMM-Corp-Site-Icons-24TMM-Corp-Site-Icons-25TMM-Corp-Site-Icons-26TMM-Corp-Site-Icons-27TMM-Corp-Site-Icons-28TMM-Corp-Site-Icons-29TMM-Corp-Site-Icons-30TMM-Corp-Site-Icons-31TMM-Corp-Site-Icons-32TMM-Corp-Site-Icons-33TMM-Corp-Site-Icons-34TMM-Corp-Site-Icons-35TMM-Corp-Site-Icons-36TMM-Corp-Site-Icons-37TMM-Corp-Site-Icons-38TMM-Corp-Site-Icons-39TMM-Corp-Site-Icons-40TMM-Corp-Site-Icons-41TMM-Corp-Site-Icons-42TMM-Corp-Site-Icons-43TMM-Corp-Site-Icons-44TMM-Corp-Site-Icons-45TMM-Corp-Site-Icons-46TMM-Corp-Site-Icons-47TMM-Corp-Site-Icons-48TMM-Corp-Site-Icons-49TMM-Corp-Site-Icons-50TMM-Corp-Site-Icons-51TMM-Corp-Site-Icons-52TMM-Corp-Site-Icons-53TMM-Corp-Site-Icons-54TMM-Corp-Site-Icons-55TMM-Corp-Site-Icons-56TMM-Corp-Site-Icons-57TMM-Corp-Site-Icons-58TMM-Corp-Site-Icons-59TMM-Corp-Site-Icons-60TMM-Corp-Site-Icons-61TMM-Corp-Site-Icons-62TMM-Corp-Site-Icons-63TMM-Corp-Site-Icons-64TMM-Corp-Site-Icons-65TMM-Corp-Site-Icons-66TMM-Corp-Site-Icons-67TMM-Corp-Site-Icons-68TMM-Corp-Site-Icons-69TMM-Corp-Site-Icons-70TMM-Corp-Site-Icons-71TMM-Corp-Site-Icons-72
Close Popup