Coronavirus disease 2019 and epidermolysis bullosa: Report of three cases.
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ABSTRACT:
SUBMITTER: Abdollahimajd F
PROVIDER: S-EPMC7435522 | biostudies-literature | 2020 Nov
REPOSITORIES: biostudies-literature
Publications
Coronavirus disease 2019 and epidermolysis bullosa: Report of three cases.
Abdollahimajd Fahimeh F Youssefian Leila L Pourani Mohammad Reza MR Vahidnezhad Hassan H Uitto Jouni J
Dermatologic therapy 20200905 6
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Project description:Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Patients with EBA suffer from chronic inflammation as well as blistering and scarring of the skin and mucous membranes. Current treatment options rely on non-specific immunosuppression, which in many cases, does not lead to a remission of treatment. Hence, novel treatment options are urgently needed for the care of EBA patients. During the past decade, decisive clinical observations, and frequent use of pre-clinical model systems have tremendously increased our understanding of EBA pathogenesis. Herein, we review all of the aspects of EBA, starting with a detailed description of epidemiology, clinical presentation, diagnosis, and current treatment options. Of note, pattern analysis via direct immunofluorescence microscopy of a perilesional skin lesion and novel serological test systems have significantly facilitated diagnosis of the disease. Next, a state-of the art review of the current understanding of EBA pathogenesis, emerging treatments and future perspectives is provided. Based on pre-clinical model systems, cytokines and kinases are among the most promising therapeutic targets, whereas high doses of IgG (IVIG) and the anti-CD20 antibody rituximab are among the most promising "established" EBA therapeutics. We also aim to raise awareness of EBA, as well as initiate basic and clinical research in this field, to further improve the already improved but still unsatisfactory conditions for those diagnosed with this condition.
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Project description:Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII collagen -- basement membrane zone antigen. Standard therapy for EBA includes a combination of systemic corticosteroids and dapsone; however, severe cases may require advanced treatment. The current article reports on four EBA cases in which biologics: infliximab, rituximab (Rtx), and intravenous immunoglobulin (IVIG) were applied. All patients fulfilled the clinical and immunological criteria of EBA: they presented tense blisters healing with atrophic scars on the skin on traumatized areas and in mucous membranes. The diagnosis of EBA was established using numerous techniques: direct and indirect immunofluorescence, salt split skin, ELISA, Fluorescence Overlay Antigen Mapping using Laser Scanning Confocal Microscopy. Since all the patients did not achieve long-term remission on standard treatment (prednisone, dapsone) due to ineffectiveness or side effects of drugs, they eventually were treated with biologics leading to extraordinary skin improvement and stopping the disease for 1-3 years. Biologics in all patients were tolerated very well. No side effects were observed during application as well as multi-month follow-up. The presented cases provide a premise that biological drugs can be a valuable component of EBA therapy.
| S-EPMC10371012 | biostudies-literature
Project description:Autoimmune response to cutaneous basement membrane components superimposed on a genetic skin fragility disease, hereditary epidermolysis bullosa (EB), has been described, but its effects on disease course remain unclear. We report a 69-year-old individual with congenital skin fragility and acral trauma-induced blistering that had suddenly worsened with the onset of severe itch and diffuse spontaneous inflammatory blisters. Next-generation sequencing identified compound heterozygous null and missense COL7A1 mutations, allowing the diagnosis of recessive dystrophic EB. However, the patient's clinical history prompted us to investigate whether he might have developed a pathological autoimmune response against basement membrane components. Tissue-bound and circulating IgG antibodies to the major bullous pemphigoid (BP) antigen, BP180, were detected in the patient's skin and serum, respectively, consistent with a diagnosis of BP. Corticosteroid therapy was initiated resulting in remission of BP manifestations. EB patients presenting rapid disease worsening should be investigated for the development of a concomitant autoimmune blistering disease.
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Project description:BackgroundThe coronavirus disease 2019, caused by severe acute respiratory syndrome coronavirus 2, is a global public health emergency. Data on the effect of coronavirus disease 2019 in pregnancy are limited to small case series.ObjectiveTo evaluate the clinical characteristics and outcomes in pregnancy and the vertical transmission potential of severe acute respiratory syndrome coronavirus 2 infection.Study designClinical records were retrospectively reviewed for 116 pregnant women with coronavirus disease 2019 pneumonia from 25 hospitals in China between January 20, 2020, and March 24, 2020. Evidence of vertical transmission was assessed by testing for severe acute respiratory syndrome coronavirus 2 in amniotic fluid, cord blood, and neonatal pharyngeal swab samples.ResultsThe median gestational age on admission was 38+0 (interquartile range, 36+0-39+1) weeks. The most common symptoms were fever (50.9%, 59/116) and cough (28.4%, 33/116); 23.3% (27/116) patients presented without symptoms. Abnormal radiologic findings were found in 96.3% (104/108) of cases. Of the 116 cases, there were 8 cases (6.9%) of severe pneumonia but no maternal deaths. One of 8 patients who presented in the first trimester and early second trimester had a missed spontaneous abortion. Of 99 patients, 21 (21.2%) who delivered had preterm birth, including 6 with preterm premature rupture of membranes. The rate of spontaneous preterm birth before 37 weeks' gestation was 6.1% (6/99). One case of severe neonatal asphyxia resulted in neonatal death. Furthermore, 86 of the 100 neonates tested for severe acute respiratory syndrome coronavirus 2 had negative results; of these, paired amniotic fluid and cord blood samples from 10 neonates used to test for severe acute respiratory syndrome coronavirus 2 had negative results.ConclusionSevere acute respiratory syndrome coronavirus 2 infection during pregnancy is not associated with an increased risk of spontaneous abortion and spontaneous preterm birth. There is no evidence of vertical transmission of severe acute respiratory syndrome coronavirus 2 infection when the infection manifests during the third trimester of pregnancy.
| S-EPMC7177142 | biostudies-literature
Project description:The 2019 novel coronavirus 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 limits opportunities to seek care. Although 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 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 epidermolysis bullosa patients of all subtypes during the coronavirus crisis.
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Project description:BackgroundVaccination is the most important measure to control the coronavirus disease 2019 (COVID-19) pandemic. Myocarditis has been reported as a rare adverse reaction to COVID-19 vaccines. The clinical presentation of myocarditis in such cases can range from mild general symptoms to acute heart failure.Case summaryWe report the cases of two young men who presented with chest pain and dyspnoea following the administration of the mRNA COVID-19 vaccine. Cardiac investigations revealed findings typical of acute myocarditis.DiscussionMyocarditis is a rare complication following mRNA COVID-19 vaccination. In this case series, the temporal proximity of the development of acute myocarditis and the administration of the mRNA COVID-19 vaccine was acknowledged. In the absence of other causative factors, myocarditis in these patients potentially occurred due to an adverse reaction to the mRNA COVID-19 vaccine. However, a causal relationship remains speculative. Clinical suspicion of myocarditis should be high if patients present with chest pain or dyspnoea after receiving COVID-19 vaccination.
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Project description:ObjectiveTo analyze the diagnosis and treatment of patients with chronic renal failure complicated with novel coronavirus pneumonia, and to evaluate the effect of blood purification technology on the treatment and prognosis of such patients.MethodsTwo COVID-19 cases undergoing hemodialysis with chronic renal failure were retrospectively analysed in our hospital.ResultsTwo COVID-19 patients were admitted to hospital due to cough, with or without fever. Laboratory tests showed decreased lymphocyte count, elevated PCT, IL-10, IL-6, TNF-α, IL-2R, high-sensitivity cardiac troponin I, NT-proBNP, creatinine, and urea nitrogen. Chest CT scan showed multiple blurred plaques and patchy shadows in both patients. Two patients received continuous venovenous hemodiafiltration (CVVHDF) every other day for 4-6 h everytime, in addition to the standard treatment. After CVVHDF, not only cytokines were reduced, but also liver function and cardiac function significantly improved. Both patients did not develop severe pneumonia. They were discharged on March 1, 2020 when meeting the discharge criteria.ConclusionTwo COVID-19 patients on maintenance hemodialysis discharged after a month of hospitalization. The removal of cytokines through blood purification technology may be beneficial for the recovery of COVID-19 patients.
| S-EPMC7191272 | biostudies-literature
Project description:Inherited epidermolysis bullosa (IEB) represents a group of rare genetic dermatoses comprising various phenotypes ranging from severe cutaneous and extracutaneous involvement to mild cutaneous fragility. Pathogenic variants have been identified in at least 20 genes responsible for IEB. In the present study, six cases of epidermolysis bullosa were recruited and subjected to a combination of clinical and genetic analysis. The family history of each case was surveyed. Whole exome sequencing was performed to identify the causative variation. The six patients showed typical EB symptoms. In all cases, WES detected the diagnostic variations of the COL7A1 or DST gene. A total of 10 variants were identified and verified. The findings of the present study further expanded the mutation spectrum of IEB, provided evidence for genetic counseling to the affected families, as well as highlighted the complexity of the pathogenesis of IEB.
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Project description:BackgroundThe lack of validated outcome measures for epidermolysis bullosa (EB) presents major barriers to evaluating disease severity and comparing the efficacy of therapies. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) was recently introduced as a valid and reliable instrument for EB; however, its interpretation for use in clinical practice and clinical trials is yet to be defined.ObjectiveTo assess the interpretability of the EBDASI in classifying patients according to disease severity and clinical response.MethodsA total of 53 outpatients with EB at two interstate institutions were prospectively evaluated. At each visit, the principal dermatologist completed the EBDASI and global assessments of disease severity and change. Classifications for mild, moderate and severe disease using the EBDASI were determined using receiver operating characteristic curves. Minimal clinically important differences for the EBDASI activity subscale were calculated and compared with the standard error of measurement.ResultsTotal EBDASI score ranges of 0-42, 43-106 and 107-506 corresponded to mild, moderate and severe disease respectively. Reduction in EBDASI activity scores of greater than 9 indicated clinically significant improvement. An increase of 3 in the activity score indicated deterioration.ConclusionThe EBDASI is a responsive tool and may be useful in characterizing disease severity and response. The cut-offs proposed in this study provide the first practical guide for interpreting the EBDASI, further supporting its use for longitudinal patient assessment and in clinical trials.
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Project description:ObjectiveTo assess feasibility of modified protocol during percutaneous tracheostomy in coronavirus disease 2019 pandemic era.DesignA retrospective review of cohort who underwent percutaneous tracheostomy with modified protocol.SettingsMedical, surgical, and neurologic ICUs.SubjectsPatients admitted in medical, surgical, and neurologic units with prolonged need of mechanical ventilation or inability to liberate from the ventilator.InterventionsA detailed protocol was written. Steps were defined to be performed before apnea and during apnea. A feasibility study of 28 patients was conducted. The key aerosol-generating portions of the procedure were performed with the ventilator switched to standby mode with the patient apneic.Measurements and main resultsData including patient demographics, primary diagnosis, age, body mass index, and duration of apnea time during the tracheostomy were collected. Average ventilator standby time (apnea) during the procedure was 238 seconds (3.96 min) with range 149 seconds (2.48 min) to 340 seconds (5.66 min). Single-use (disposable) bronchoscopes (Ambu A/S [Ballerup, Denmark] or Glidescope [Verathon, Inc., Bothell, WA]) were used during all procedures except in nine. No desaturation events occurred during any procedure.ConclusionsPercutaneous tracheostomy performed with apnea protocol may help minimize aerosolization, reducing risk of exposure of coronavirus disease 2019 to staff. It can be safely performed with portable bronchoscopes to limit staff and minimize the surfaces requiring disinfection post procedure.
| S-EPMC7259562 | biostudies-literature