Skin Manifestations of VEXAS Syndrome and Associated Genotypes.

Importance: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined genetic disease with an estimated prevalence of 1 in 4269 men older than 50 years and is marked by systemic inflammation, progressive bone marrow failure, and inflammatory cutaneous manifestations. Objective: To define the spectrum of cutaneous manifestations in VEXAS syndrome and the association of these findings with clinical, genetic, and histological features. Design, Setting, and Participants: This observational cohort study included data from 112 patients who were diagnosed with VEXAS-defining genetic variants in UBA1 between 2019 and 2023. Data were collected from medical record review or from patients with VEXAS directly evaluated at the National Institutes of Health in Bethesda, Maryland. Main Outcomes and Measures: To define the spectrum of cutaneous manifestations in VEXAS in association with genetic, histological, and other clinical findings. A secondary outcome was cutaneous response to treatment in VEXAS. Results: Among the 112 patients (median [range] age, 69 [39-79] years; 111 [99%] male), skin involvement was common (93 [83%]), and the most frequent presenting feature of disease (68 [61%]). Of 64 histopathologic reports available from 60 patients, predominant skin histopathologic findings were leukocytoclastic vasculitis (23 [36%]), neutrophilic dermatosis (22 [34%]), and perivascular dermatitis (19 [30%]). Distinct pathogenic genetic variants were associated with specific cutaneous manifestations. The p.Met41Leu variant was most frequently associated with neutrophilic dermal infiltrates (14 of 17 patients [82%]), often resembling histiocytoid Sweet syndrome. In contrast, the p.Met41Val variant was associated with vasculitic lesions (11 of 20 patients [55%]) with a mixed leukocytic infiltrate (17 of 20 patients [85%]). Oral prednisone improved skin manifestations in 67 of 73 patients (92%). Patients with VEXAS treated with anakinra frequently developed severe injection-site reactions (12 of 16 [75%]), including ulceration (2 of 12 [17%]) and abscess formation (1 of 12 [8%]). Conclusions and Relevance: Results of this cohort study show that skin manifestations are a common and early manifestation of VEXAS syndrome. Genetic evaluation for VEXAS should be considered in older male patients with cutaneous vasculitis, neutrophilic dermatoses, or chondritis. Awareness of VEXAS among dermatologists is critical to facilitate early diagnosis.

Virtual conferences and e-learning in dermatology during COVID-19 pandemic: Results of a web-based, global survey.

During the pandemic, virtual conferences became the norm. We conducted a cross-sectional global study to assess dermatologists' responses to virtual conference and e-learning program attendance, as well as to discuss the status of such events during the pandemic. This web-based, global survey included 733 dermatologists. Primary outcomes are percentages of responders answering questions. Assessment of the relationship between two categorical variables was performed with the chi-square test. A substantial percentage of responders were willing to attend a virtual meeting (70.6%) or webinar (80.2%), or to conduct a webinar (47.3%). Among participants who provided resident or fellow training before the pandemic, 38% responded that they did not have any teaching systems in place during the pandemic. Virtual conference attendance was significantly associated with video conference attendance before the pandemic, webinar attendance, teledermatology (TD) use during the pandemic, future TD use, having training systems in place for residents or fellows (P < .001 for each), and North American location of participant (P = .001). Webinar attendance was associated with North American location, conducting webinars (P < .001 for each), and future TD use (P = .024). This pandemic has had a profound effect on dermatology conferences and e-learning programs. Attending video conferences and webinars or other online training was associated with TD use and future use, which indicates that these technologies are all here to stay.

Global impact on dermatology practice due to the COVID-19 pandemic.

This contribution focuses on the effects of coronavirus disease 2019 (COVID-19) on dermatology practice. We discuss the impact on practice volume and procedures and on the considerable increase in teledermatology use. We also describe the important roles that dermatologists have played in enhancing infection prevention and on the frontline. During the crisis, dermatologists have faced the challenge of a shortage of resources, such as personal protective equipment, in the health care system. In addition, they have been involved in managing cutaneous manifestations related to COVID-19 and occupational disease caused by personal protective equipment. Dermatologists have made a diligent effort to identify melanoma and to ensure the treatment of high-risk skin cancers. Safety guidelines have been suggested to minimize the potential risks associated with the systemic use of immunosuppressant agents and immunomodulators in patients with severe inflammatory skin disease during the pandemic. Finally, social distancing necessitated that dermatology conferences take place virtually and teaching via e-learning increased.