key: cord-0790875-sr6x4y4h authors: Ramirez-Quizon, Mae; Murrell, Dédée F. title: Managing epidermolysis bullosa during the COVID-19 pandemic: experience and ideals date: 2021-02-01 journal: Clin Dermatol DOI: 10.1016/j.clindermatol.2021.01.014 sha: e5207d7067d67efe0ed96b4922f56d927caada3e doc_id: 790875 cord_uid: sr6x4y4h The 2019 novel coronavirus (COVID-19) pandemic has tremendously affected health-seeking behaviors. Fear of contracting the disease has been a major factor keeping patients from presenting to hospitals, even when urgent or emergent medical attention is needed. Hospitals limiting staff exposure and capacity to accommodate patients also limit opportunities to seek care. While physical distancing is encouraged to curb infections, this call needs to be tempered with public health education for what constitutes emergencies and urgent medical conditions needing face-to-face attention. Measures to assuage fears among patients and their caregivers to ensure their safety in the hospital or health care setting need to be communicated and executed effectively. Epidermolysis bullosa (EB) is an inherited mechanobullous disorder that is usually stable, but in some patients with underlying comorbidities, close monitoring or face to face management is required . We present our experience and provide recommendations pertinent to EB patients of all subtypes during the COVID-19 crisis. Epidermolysis bullosa (EB) is an inherited mechanobullous disorder that is usually stable, but in some patients with underlying comorbidities, close monitoring or face to face management is required . We present our experience and provide recommendations pertinent to EB patients of all subtypes during the COVID-19 crisis. -- The extent to which countries and governments around the world have instituted drastic measures to curb the transmission of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) which causes the 2019 novel coronavirus (COVID-19) is unprecedented. While frequent handwashing, cough etiquette, and mask-wearing are common advice to avoid contracting the virus short of testing, physical distancing is at the core of these measures and has greatly restricted movement in at least the busiest parts of the globe. Whether government mandates a total lockdown or an individual selfquarantines with social distancing, calls to scale back physical interaction have affected healthcare-seeking behavior 123 . Emergency and urgent care centers that would otherwise have been busy before the coronavirus pandemic have reported a record-low volume of patient encounters. While the decrease in emergent and even non-emergent consults may be a relief for any area that expects a surge in COVID-19-related consults, it becomes a concern when a significant percentage of patients who have lifethreatening medical issues are not presenting to hospitals. This brings to the fore the issue of fear of contracting the virus among patients, should they physically consult at hospitals or even outpatient doctors' clinics or health centers 4 . Some patients are afraid to seek or accept medical help at all for even lifethreatening conditions due to fear of contracting COVID-19, with all of the suffering and isolation that goes with being infected. A group has embarked on an initiative to allay fears and improve the perception of risk in an emergency department in the United States by targeted messaging about what constitutes an emergency along with information about concrete measures taken by the hospital to ensure patient safety 4 . Many other hospitals around the world have employed a similar approach to encourage patients to address urgent or emergent health concerns by seeking medical care as soon as possible 5678 . Whether these efforts translate to tangible, measurable improvement in morbidity and mortality has yet to be determined. This scenario, however, is not unique to emergency departments with highacuity patients. Even those who have chronic, generally stable conditions like epidermolysis bullosa (EB) may have underlying problems that may otherwise have been caught early or would have received earlier intervention if not for fear of presenting to the hospital. EB is an inherited mechanobullous disorder with marked mechanical fragility of epithelial tissues, resulting in blistering and erosions that may follow even trivial trauma 9 . Four major types are recognized: Chronic wounds from EB are a major concern, and when occurring with other comorbidities can enhance susceptibility to COVID-19. There is now an algorithm for care of a variety of patients with chronic wounds during the pandemic 11 . SARS-CoV-2 enters host cells using its spike protein to bind to the cell receptor angiotensin converting enzyme 2 (ACE2). It is known to be expressed not only in the mucosa such as the nasal epithelium but also in the capillaries of the skin, basal layer of keratinocytes, and eccrine glands 12 . It has been posited that any skin wound deep enough to expose the basal epidermis is a potential entry point for the virus 13 . In addition, it has been suggested that skin damage caused by irritant hand dermatitis from frequent handwashing provides a route of entry for the SARS-CoV-2 virus 14 . A type of bronchial progenitor cell co-expressing both ACE2 and cofactor transmembrane protease serine 2 (TMPRSS2) 15 may be a more likely target of SARS-CoV-2 than open wounds 16 . The SARS-CoV-2 is primarily spread via droplet and is not bloodborne; no evidence exists as yet to support the claim that it is transmitted via skin wounds 1718 . Despite this, syndromic forms of EB may be associated with significant morbidity and mortality 1920 with involvement of organ systems other than the skin that require regular follow-up with a multidisciplinary team. The phenotype of 3 individuals with EB from 2 families who contracted COVID-19 has been recently described. The two women with RDEB p from a family with four EB patients have had disparate courses of illness. The 32-year-old younger sister has had a milder course, while the 35-year old sister was hospitalized due to the severity of manifestations that were comparable to those seen in the general population. These later resolved after 8 days of hospitalization. The third patient, a 35-year-old syndromic EBS, presented with mild signs of infection that spontaneously resolved after 1 week. While these cases are too few to extrapolate findings to all EB patients, it does provide a very helpful account: EB and EB-related complications, such as anemia, esophageal strictures, growth retardation, or nephropathy, do not guarantee a severe course. More studies will answer questions about the prevalence and outcome of EB patients with COVID-19 19 . Measures taken by hospitals or in physician offices to reduce patient load staff exposure to potential COVID-19 patients may have limited the options for those who are willing to seek care. DFM has encountered a patient with junctional EB on hemodialysis who developed pericarditis with a large pericardial effusion that required drainage. Due to lack of human resources for an outpatient echocardiography, the procedure could not be performed unless the patient was admitted as an inpatient. Other severe RDEB and JEB patients have been declining their in-person reviews of their wounds, placing them at risk for developing aggressive squamous cell carcinoma. One patient has even been refusing funded EB nursing visits to the home, solely for changing dressings, for fear of contracting COVID-19. This has continued despite the very low community transmission of SARS-CoV-2 in the state of NSW where Sydney is the state capitol. To our knowledge, no Australian EB patient has contracted COVID-19. This highlights the many obstacles an EB patient may encounter amidst the pandemic. MRQ reports even fewer EB patients seeking direct care in dermatology centers other than virtually in the Philippines. As of this writing, the Philippines has the highest prevalence of COVID-19 cases in the Western Pacific Region at nearly 300,000 cases 21 . Most patients with mild to moderate EB will have chronic wounds. It is not clear whether the profile of EB patients who consult is influenced by early mortality of the patients with more severe EB types in this country. An international panel of EB experts which includes the authors has published a consensus on the multidisciplinary approach to the care of EB patients during the COVID-19 pandemic (Table 1) 2223 . Other recommendations for the EB patients, caregivers, and health workers have also been published 202425 . While EB patients do not seem to be at an increased risk of contracting COVID-19 as compared to the general population, those with significant internal complications may have secondary risk factors that weaken their ability to fight infection, COVID-19 included. General precautions apply, such as staying home and limiting non-essential travel and ensuring an adequate stockpile of supplies for EB care such as dressings 26 . Open communication lines between the EB specialist or team is also vital. Hand dressings worn by EB patients to cushion mechanical injury preclude frequent handwashing as recommended by the WHO 27 . The 20-second rubbing together of skin with soap and water in a patient with inherent skin fragility affects compliance, as does the preferred antisepsis of rubbing the hands with an alcohol-based formulation. This occurs even if this has been found to be more effective at killing lipophilic enveloped viruses like SARS-CoV-2 in vitro and in vivo, more time-saving, and easier to use 28 . An outer layer of dressings or bandages that is changed frequently is an acceptable alternative in patients who cannot perform these manners of hand hygiene 20 . Personal protective equipment (PPE) should be worn by patients as well as all nursing and medical staff when attending to EB patients. EB patients should apply protective dressings to areas of skin where the mask may rub on their skin. In addition, they should apply a bland moisturizer to their facial skin prior to donning the mask in order to reduce the friction of the mask on their skin 22 . Local PPE recommendations, according to local prevalence rates and availability of PPE should be followed 29 . (Figure 1 During the pandemic, instead of transferring neonates with EB to regional EB centers, these infants should be cared for in their local hospitals with virtual guidance from the regional EB multidisciplinary team. This group of EB patients who are seen at 6 to 12 months should be managed vitually. This permits the various MDT members to communicate together about their care. This group of EB patients is typically reviewed in person by the MDT every 3 months. Instead, if their blood tests and any other scan results are available ahead of time, a virtual MDT should occur in advance of the visit in which there is communication with the patient and his/her care giver. The leaders of the team, such as the EB dermatologist and EB nurse, could perform this review along with any other relevant team members based on the current problem list. It is more difficult to examine the whole skin using telehealth in this highrisk group and ideally this should be done face to face. Sometimes, the SCCs just look like normal wounds, but the pain quality or intensity may differ from the usual wounds. The patient, care giver, and the clinical team should all wear masks and appropriate PPD> Because EB patients are so vulnerable, any EB specialist nurse or part time nurse should not care for an EB patient if working or having done any shift in a hospital or other healthcare facility where there have been active COVID 19 patients. Such workers should not have visited any hotspot areas or have had contact with any infected relatives or friends. If an EB patient contracts COVID-19, he or she should ideally have a private room. Such patients are especially vulnerable and require open wounds to be redressed every 1-2 days. Anticoagulation therapy should be continued . The COVID-19 pandemic has led to a decline in face to face consults, even in patients with syndromic EB who may have underlying problems that require monitoring or urgent intervention. We present our experience and recommendations based on an international consensus of EB experts that was coordinated by DFM and recently published 22 . Anecdotal advice is offered in light of a paucity of information on the challenges and issues faced by EB patients in these unprecedented times with the hope of decreasing preventable morbidity or even mortality in this unique subset of patients. Chart of the week: The alarming drop in referrals from GPs to hospital services since the Covid-19 outbreak The impact of the COVID-19 pandemic on cancer deaths due to delays in diagnosis in England, UK: a national, population-based, modelling study The impact of the COVID-19 epidemic on the utilization of emergency dental services Where Are All the Patients? 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