Publications by authors named "Moise Levy"
Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic disorder due to pathogenic variants in the COL7A1 gene. In this study we determined the association between different categories of COL7A1 variants and clinical disease severity in 236 RDEB patients in North America. Published reports or in-silico predictions were used to assess the impact of pathogenic variants in COL7A1 on type VII collagen (C7) protein function.
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- Encephalocraniocutaneous lipomatosis (ECCL), or Haberland syndrome, is a rare condition affecting the skin, central nervous system, and eyes, characterized by tumors and other abnormalities.
- Key symptoms include specific skin lesions, subcutaneous lipomas, and neurological issues like intracranial lipomas and developmental delays.
- Diagnosis primarily relies on clinical evaluation with possible genetic testing; management is multidisciplinary, focusing on symptom relief and monitoring for potential complications.
Background: Congenital ichthyoses (CI) comprise a heterogeneous group of genetic diseases requiring lifelong treatment and having a major effect on quality of life. Conventional treatments reduce scaling and skin discomfort; however, they usually have little or no effect on erythema and pruritus. The identification of cytokine alterations in CI raised the possibility of repurposing available biologics.
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- * Mild-to-moderate AD is typically treated with topical anti-inflammatory medications and basic skin care, while moderate-to-severe cases often need systemic therapy, including off-label immunosuppressants, which carry safety concerns.
- * New targeted biologics and small molecules are emerging as more effective and safer treatment options for AD, leading to the development of standardized recommendations for treating pediatric patients with moderate-to-severe cases.
Article Synopsis
- The study focused on the challenges of transitioning patients with epidermolysis bullosa (EB) from pediatric to adult care, revealing that provider support is crucial during this process.
- A survey conducted with members of the Epidermolysis Bullosa Clinical Research Consortium found that 88% of providers were concerned about this transition, while only 38% had formal transition of care (TOC) programs.
- The results highlighted the importance of establishing formal TOC programs and a team-based approach, emphasizing the need to identify adult care providers to facilitate better transitions for patients.
Article Synopsis
- Pediatric atopic dermatitis has seen a rise in emergency visits and healthcare costs over the last 20 years, highlighting a need for better clinician education on this condition.
- A quality improvement initiative recruited 56 clinicians in Travis County, Texas, aiming to enhance their knowledge and confidence in diagnosing and managing pediatric atopic dermatitis through surveys, quizzes, and multimodal education.
- Results showed significant improvements in quiz scores (average increase of 10% overall and 37% for treatment-specific knowledge) and clinician confidence, indicating that the program is an effective resource for training healthcare providers in both emergency and outpatient settings.
Article Synopsis
- - The study focuses on creating standardized best practices for managing Epidermolysis bullosa (EB) in hospitals, as current training is inadequate and lacks unified guidelines in North America.
- - A modified Delphi method was used to gather expert opinions from dermatologists, nurses, and caregivers, resulting in consensus agreements on treatment practices for both neonatal and postneonatal patients with EB.
- - The final consensus involved 103 neonatal and 105 postneonatal statements, aiming to enhance inpatient care quality for EB patients, with a note that recommendations might need to be tailored to individual cases.
Posterior reversible encephalopathy syndrome in the setting of IgA vasculitis.
Pediatr Dermatol
July 2024
Article Synopsis
- IgA vasculitis (IgAV) is a small vessel inflammation mainly affecting kids and can impact organs like the kidneys, lungs, and brain.
- A key complication in the brain is posterior reversible encephalopathy syndrome (PRES), characterized by high blood pressure, confusion, and seizures due to blood-brain barrier issues.
- The article discusses a rare case of PRES as an initial sign of IgAV to highlight this uncommon connection and improve awareness among medical professionals.
Article Synopsis
- A 20-month-old girl with Schimmelpenning-Feuerstein-Mims (SFM) syndrome, affecting her skin on the head, neck, and torso, was treated successfully with topical trametinib.
- Trametinib targets the MAPK signaling pathway, which includes the mutated KRAS gene found in the child's condition, marking a novel use for the medication.
- Over 16 months of applying trametinib twice daily, the patient experienced relief from itching and reduction in the size of skin plaques.
Article Synopsis
- Ichthyoses are skin disorders marked by scaling and redness, leading to the creation of the Ichthyosis Scoring System (ISS) to quantify their severity effectively.
- A study involving 65 participants found that ISS had excellent reliability for scoring skin conditions, both through live evaluation and photographic images.
- The results indicate that ISS is a valid and reliable tool for assessing ichthyosis severity, making it suitable for clinical use and potentially a standard in the field.
Article Synopsis
- Medical procedures can cause significant pain and anxiety in patients, potentially leading to long-term negative effects if not managed properly.
- A study aimed to compare the effectiveness of virtual reality (VR) headsets and noise-canceling headphones (HP) against standard treatment (TAU) in alleviating pain and anxiety for children undergoing dermatology procedures.
- Results showed that both VR and HP significantly reduced anxiety levels from before to after the procedures, whereas the TAU group did not experience the same reduction, highlighting the potential benefits of technology-based interventions in pediatric care.
Article Synopsis
- The most frequently found bacteria in wound cultures of Epidermolysis Bullosa patients are Staphylococcus aureus and Pseudomonas aeruginosa.
- Researchers focused on analyzing patients with wound cultures that tested positive for Pseudomonas aeruginosa due to its prevalence and potential link to cancer development.
- The study aims to provide insights for future research and improvement in wound care strategies for those with Epidermolysis Bullosa.
There are little published data on the transition of care in EB. We conducted a survey study recruiting EB patients from the Dystrophic EB Research Association (debra) website and centers caring for high numbers of EB patients in the United States and internationally from Sept 17, 2019 to Nov 3, 2021. The majority of participants had not discussed the transition of care with their healthcare providers, nor the healthcare needs to be required as an adult.
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- The study focuses on caregiver satisfaction and experiences with G-tubes in children with epidermolysis bullosa (EB), highlighting the benefits of this nutritional support intervention despite initial parental apprehension.
- Conducted through an anonymous 89-item online survey among caregivers from nine institutions, the findings revealed that most caregivers reported high satisfaction levels (90%) post-G-tube placement and some wished they had adopted it earlier.
- Results indicate improved mealtime experiences for both caregivers and children with EB after G-tube insertion, suggesting that caregiver feedback can guide future research and practices in managing G-tubes.
Ocular Melanocytosis and Peripheral Retinal Avascularity in a Case of Cutis Marmorata Telangiectatica Congenita.
Ophthalmic Surg Lasers Imaging Retina
November 2022
Article Synopsis
- - Acne is a common condition that affects most people, especially during adolescence, with a rising prevalence noted in preadolescents aged 7 to 12 years.
- - Recent developments in acne treatments since the 2016 guidelines necessitate that primary care physicians stay updated on new management strategies, particularly for younger patients.
- - The 2019 Preadolescent Acne Roundtable identified five key areas for improvement in treating acne, emphasizing the importance of communication regarding hormones, psychological factors, skin color considerations, and evaluating treatment effectiveness.
Article Synopsis
- Atopic dermatitis (AD) is a widespread chronic condition in children, often managed by primary care providers, but sometimes requiring dermatology specialists.
- A survey of 50 caregivers revealed that while they felt both PCPs and specialists listened to their concerns, many believed that specialists offered better care for managing AD.
- The study suggests that earlier and more frequent referrals to dermatology specialists might benefit children with AD, indicating a need for further research on access to care based on race and ethnicity.
Article Synopsis
- The study aimed to evaluate how reliable and valid remote assessments of atopic dermatitis severity are, using caregiver-provided photos (p-EASI) and videos (v-EASI) compared to traditional in-person evaluations.
- A group of 50 children with varying severity of atopic dermatitis had their skin condition assessed by caregivers through photos and videos, and by clinicians using in-person ratings.
- Results showed that p-EASI demonstrated strong reliability and validity, while v-EASI had moderate reliability, indicating both methods could be useful for assessing atopic dermatitis remotely.
Article Synopsis
- Dermatologists are skilled in treating complex skin conditions but may lack expertise in addressing the psychosocial aspects of care for pediatric patients with epidermolysis bullosa (EB).
- The article highlights the importance of recognizing various types of trauma—social, psychological, and medical—that children with EB may face.
- It provides recommendations for trauma-informed care and emphasizes the need for a patient-centered approach in managing anxiety and trauma in these patients, involving both dermatologists and multidisciplinary teams.
Article Synopsis
- Accurate diagnosis of epidermolysis bullosa (EB) is crucial for effective treatment and counseling, as different types require different management strategies.
- The study analyzed testing patterns among 771 patients, comparing results from transmission electron microscopy (TEM), immunofluorescence mapping (IFM), and genetic analysis to determine how often they produced consistent diagnoses.
- Findings showed a preference for genetic analysis over traditional methods, but both TEM and IFM often yielded unclear or inconsistent results, indicating the need for a more definitive diagnostic approach.
Reframing racial and ethnic disparities in atopic dermatitis in Black and Latinx populations.
J Allergy Clin Immunol
November 2021
Article Synopsis
- Black people in the U.S. have higher rates and severity of atopic dermatitis (AD) compared to White people, while the Latinx population shows less researched but notable differences in AD characteristics.
- Genetic factors linked to AD risk appear less prevalent among Black individuals, suggesting that social and environmental issues may play a larger role in these disparities.
- Emphasizing socioeconomic, environmental, and healthcare factors influenced by racism is crucial to understanding and addressing the differences in AD prevalence and severity among racial and ethnic groups, rather than focusing solely on genetic explanations.