Dedee F Murrell1, Anne W Lucky2, Julio C Salas-Alanis3, David T Woodley4, Francis Palisson5, Ken Natsuga6, Milos Nikolic7, Mae Ramirez-Quizon8, Amy S Paller9, Irene Lara-Corrales10, Mohammadreza Amir Barzegar11, Eli Sprecher12, Cristina Has13, Martin Laimer14, Anna L Bruckner15, Asli Bilgic16, Arti Nanda17, Diana Purvis18, Alain Hovnanian19, Slobodna Murat-Sušić20, Johannes Bauer14, Johannes S Kern21, Christine Bodemer22, Linda K Martin23, Jemima Mellerio24, Cezary Kowaleski25, Susan J Robertson26, Leena Bruckner-Tuderman13, Elena Pope10, M Peter Marinkovich27, Jean Y Tang27, John Su28, Jouni Uitto29, Lawrence F Eichenfield30, Joyce Teng27, Mark Jean Aan Koh31, Sang Eun Lee32, Phuong Khuu27, Heather I Rishel33, Mette Sommerlund34, Karen Wiss35, Chao-Kai Hsu36, Tor Wo Chiu37, Anna E Martinez38. 1. Department of Dermatology, St George Hospital, University of New South Wales, Sydney, New South Wales, Australia. Electronic address: d.murrell@unsw.edu.au. 2. Cincinnati Children's Epidermolysis Bullosa Center, Cincinnati Children's Hospital, Cincinnati, Ohio. 3. DebRA (Dystrophic Epidermolysis Bullosa Research Association) Mexico, Monterrey, Mexico. 4. Department of Dermatology, University of Southern California, Los Angeles, California. 5. Dystrophic Epidermolysis Bullosa Research Association DebRA (Dystrophic Epidermolysis Bullosa Research Association) Chile; Clinica Alemana, Universidad del Desarrollo, Santiago, Chile. 6. Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. 7. Deptartment of Dermatovenereology, University of Belgrade School of Medicine, Belgrade, Serbia. 8. Department of Dermatology, University of the Philippines, Philippines General Hospital, Manila, Philippines. 9. Departments of Dermatology and Pediatrics, Children's Hospital, Northwestern University, Chicago, Illinois. 10. Section of Dermatology, Hospital for Sick Children, Toronto, Ontario, Canada. 11. Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 12. Department of Dermatology, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. 13. Department of Dermatology, Medical Center-University of Freiburg, Freiburg, Germany. 14. EB Haus, Department of Dermatology, Paracelsus University, Salzburg, Austria. 15. Pediatric Dermatology Department, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado. 16. Department of Dermatology, Akdeniz University, Antalya, Turkey. 17. Pediatric Dermatology Unit, As'ad Al-Hamad Dermatology Center, Kuwait. 18. Department of Dermatology, Starship Children's Health, Auckland, New Zealand. 19. Department of Genetics, Institut National de la Santé et de la Recherche Médicale Unité Mixte de Recherche 1163, Laboratory of Genetic Skin Diseases, Paris, France; Institut des Maladies Génétiques (IMAGINE), University of Paris, Paris, France. 20. Department of Dermatovenereology, University Hospital Centre, Zagreb, Croatia. 21. Dermatology Department, The Royal Melbourne Hospital, Melbourne, Victoria, Australia; The University of Melbourne, Parkville, Victoria, Australia. 22. Department of Dermatology, Necker Enfants Malades Hospital, University of Paris, Paris, France; Reference Centre for Genodermatoses and Rare Skin Diseases (MAGEC), Paris, France. 23. Department of Dermatology, Sydney Children's Hospital, University of New South Wales Faculty of Medicine, Sydney, New South Wales, Australia. 24. Adult Epidermolysis Bullosa Service, St John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom. 25. Department of Dermatology and Immunodermatology, University of Warsaw, Warsaw, Poland. 26. Dermatology Department, The Royal Melbourne Hospital, Melbourne, Victoria, Australia; Department of Dermatology, The Royal Children's Hospital, Melbourne, Victoria, Australia. 27. Department of Dermatology, Stanford University Medical Center, Palo Alto, California. 28. The University of Melbourne, Parkville, Victoria, Australia; Monash University, Eastern Health, Melbourne, Victoria, Australia. 29. Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania; Sidney Kimmel Medical College, Philadelphia, Pennsylvania. 30. Departments of Dermatology and Pediatrics, University of California, San Diego, California; Department of Pediatric Dermatology, Rady Children's Hospital, San Diego, California. 31. Dermatology Service, KK Women's & Children's Hospital, Singapore. 32. Department of Dermatology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea. 33. Rishel Pediatric Dermatology, PC, Rishel Enterprises, LLC, San Francisco, California. 34. Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark. 35. Department of Dermatology, University of Massachusetts Medical School, Worcester, Massachusetts. 36. Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan. 37. Chinese University of Hong Kong, Hong Kong, China. 38. Paediatric Dermatology Department, Great Ormond Street National Health Service Foundation Trust, London, United Kingdom.
To the Editor: The 2019 novel coronavirus (COVID-19) pandemic became apparent in China during the International Congress on Epidermolysis Bullosa (EB) in London, in January 2020. Many patients with EB have medical problems that make them a vulnerable population of patients. We developed an international consensus to suggest the best management of patients with EB during the pandemic.The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus enters host cells using its spike protein binding to the cell receptor angiotensin converting enzyme 2 (ACE2), which is expressed in several tissues. Mucosae have high ACE2 expression, particularly the nasal epithelium. ACE2 is also expressed in the basal layer of keratinocytes and sebaceous glands of normal skin as well as in vascular endothelial cells, but its expression in wounded EB skin has not been studied.A questionnaire was drafted by an author (D.M.) into a table of suggested modifications to the management of EB during the COVID-19 pandemic. Fifty-seven well-known experts on EB were selected based on membership of the international Clin-et group or clinical expertise in EB, or both, demonstrated at International EB Congress participation. Responses and reasons for each response were requested individually to the lead author based on an ideal scenario, rather than what actually may happen in some centers with financial constraints. A priori, consensus was considered to be the agreement of more than 70% of respondents with the suggestion. Questionnaires were returned by 44 of the 57 EB experts, representing several areas of clinical expertise in EB (dermatology, pediatrics, internal medicine, and surgery) from 5 continents. After addition and revision of some items and 3 cycles of revoting, consensus was achieved for all items, which are summarized in Supplementary Table I (available via Mendeley at https://data.mendeley.com/datasets/zmpncb6zpr/2).The main change in usual practice was the introduction of photographs from the patient/family and teledermatology as the primary visit for patients with less severe EB, with dressing supplies sent to the patients directly. For those patients with EB with significant internal disease, monitoring tests (blood and urine) must continue but can be obtained by local laboratories or family doctors close to home. If telehealth images are insufficient to assess lesions, assessments should be conducted at the EB center.One of the greatest fears of families caring for patients with severe forms of EB is how they will be perceived on admission to hospitals, especially institutions with limited resources, including ventilators. Because patients with EB often appear frail and emaciated, health care workers unfamiliar with the condition may underestimate their resilience and incorrectly assume that they have a low likelihood of survival. If a patient with EB required anticoagulation to manage COVID-19, there might be additional bleeding from the skin or mucosae, but blood transfusions will compensate for this. Supplemental Table I details protection for the skin and mucosae that is required for wearing masks and ventilation.