VEXAS syndrome: A review of cutaneous findings and treatments in an emerging autoinflammatory disease.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic mutation) syndrome is a novel autoinflammatory, late-onset, disorder first identified in 2020. It is caused by mutations in the UBA1 gene. The most prominent clinical features reported by VEXAS patients are cutaneous and haematological, having characteristic skin features reported as the initial presenting findings of the disease. VEXAS is a severe and treatment-resistant condition with high morbidity and mortality rates. Here, we examine all case reports and case series of VEXAS syndrome through March 2023 focusing on those presenting cutaneous manifestations. We discuss these manifestations and their reported treatment strategies. In many cases, it might be first suspected and diagnosed by dermatologists, highlighting their vital role in initiating timely multidisciplinary care.

Pyoderma gangrenosum and impact on quality of life: A narrative review.

Pyoderma gangrenosum (PG) is an autoinflammatory disorder typically characterized by progressive ulcers with dense neutrophilic infiltrates in the absence of infectious causes. The chronic nature of this disease significantly impacts the patients' quality of life (QoL). Yet there is currently a dearth of information in the literature regarding standardised treatment guidelines and the impact of PG on patients' QoL. We conducted a literature search on PubMed using the terms "pyoderma gangrenosum" AND "quality of life." We identified nine relevant articles that provide insight into which domains are affected and what treatment can improve QoL. The most common domains involved are physical, emotional, and psychological. Patients tend to feel depressed/anxious, isolated, and embarrassed secondary to PG manifestations. Comorbidities such as Crohn's disease, monoclonal gammopathy of dermatologic significance, and ulcerative colitis can worsen the impact on these patients' QoL. Pain is also a significant contributor to decreasing patients' QoL. Treatments such as topical steroids, adalimumab, and canakinumab may help improve QoL scores. We believe this information can help clinicians guide the care of patients with PG and highlight the need for more studies and clinical trials focusing on PG treatments' impact on QoL.

Generalized pustular psoriasis-like widespread eruption following COVID-19 infection in a patient with spondyloarthropathy on adalimumab.

Generalized pustular psoriasis (GPP) is a form of pustular psoriasis that is distinguished by recurring or persistent outbreaks of non-acral primary sterile pustules. These eruptions can occur with or without systemic inflammation. Various factors, such as medications, stress and viral infection, have been identified as potential triggers for GPP flares. While several cases have detailed GPP-like eruptions in the setting of coronavirus disease 2019 (COVID-19) infection, few have explored the interplay between infection and biologic use in the development of GPP. In this case, we detail the history and management of a 45-year-old male patient with a prior history of spondyloarthropathy managed on a tumour necrosis factor-α inhibitor and recent COVID-19 infection presenting with a new, spreading pustular rash.

A focused review of visible light therapies for vitiligo.

BACKGROUND: Vitiligo can be challenging to treat and exhibit an unpredictable clinical course. Phototherapy in the form of visible light can achieve both repigmentation and depigmentation outcomes in vitiligo, with minimal associated adverse events. This review focuses on the mechanistic understandings and clinical outcomes of visible light-based treatments for vitiligo. METHODS: Articles were retrieved from PubMed starting from May 1965 until August 2023, yielding 496 unique articles. We conducted title, abstract, and full-text screening to identify articles describing the use of visible light (380-750 nm), either as part of combination therapy or as monotherapy, for repigmentation or depigmentation treatment in vitiligo. RESULTS: Twenty-seven articles met inclusion criteria, offering preclinical and clinical data regarding the utilization of helium-neon laser (red light) and blue light-emitting diodes (LEDs) as methods of repigmentation therapy in vitiligo. Preclinical and clinical data on the utilization of Q-switched ruby laser (694 nm) and frequency-doubled (FD) Nd:YAG laser (532 nm) for vitiligo depigmentation therapy were also identified. CONCLUSION: While limited by small studies and a lack of standardized administration of phototherapy, the evidence for visible light's effectiveness in managing vitiligo is encouraging. Red light therapy using He-Ne lasers and blue light therapy via LEDs can stimulate repigmentation in patients with vitiligo with minimal adverse events. Q-switched ruby and FD Nd:YAG lasers provide viable, visible light depigmentation options, either alone or with topical agents. With limited clinical data, larger studies are needed to validate the efficacy of visible light therapy in treating vitiligo and to better understand its long-term outcomes.

Trends in sex and ethnicity among U.S. dermatopathology and Mohs surgery trainees: 2011-2021.

Dermatology has been cited as the second-least racially diverse medical specialty in the United States. In the last decade, the American Academy of Dermatology (AAD), the Skin of Color Society (SOCS), the Dermatology Section of the National Medical Association (NMA), and other stakeholders have made significant efforts to increase diversity in dermatology. This study aims to explore the potential impact of these efforts by analyzing sex and ethnic trends in ACGME-accredited dermatology fellowships; Mohs surgery, and dermatopathology, using data from 2011-2021. Our findings reveal that over the last decade, significant strides to increase sex diversity within dermatology have led to a growing number of female resident trainees (62%). This trend is also reflected in Mohs surgery (50%) and dermatopathology (52%) fellowships. In addition, the proportion of Underrepresented in medicine (URiM) fellowship trainees has also increased significantly over the last decade, with a now similar proportion of URiM trainees between dermatology residency, Mohs surgery, and dermatopathology.

Relatlimab-nivolumab: A practical overview for dermatologists.

BACKGROUND: Relatlimab is a human anti-lymphocyte activation gene 3 protein antibody approved for the treatment of metastatic or unresectable melanoma in combination with nivolumab, an existing programmed cell death protein 1 inhibitor. OBJECTIVE: In this article, we review the clinical literature on the efficacy and therapeutic use of the immune checkpoint inhibitor relatlimab in combination with nivolumab for metastatic melanoma. METHODS: We provide an overview of the mechanism of action, clinical efficacy, and safety profile of relatlimab-nivolumab through a review of recent publications on this emerging therapeutic combination. Ongoing clinical trials studying the use of relatlimab and associated areas of active investigation are also highlighted. CONCLUSION: This review strives to inform practicing dermatologists on the use of relatlimab-nivolumab as an approved first-line dual checkpoint inhibitor for metastatic melanoma in appropriate clinical cases.

Clinical music interventions and music therapy in dermatology.

Music interventions in medicine have been shown to reduce anxiety and depression, decrease pain, and improve quality of life; however, a review of clinical music interventions in dermatology is lacking. Studies have shown that playing music for patients undergoing dermatologic procedures (Mohs surgery and anesthetic injections) can decrease pain and anxiety. Patients with pruritic conditions-such as psoriasis, neurodermatitis, atopic dermatitis, contact eczema, and situations requiring hemodialysis-have exhibited decreased levels of disease burden and pain when listening to preferred music, pre-chosen music, and live music. Studies suggest that listening to certain types of music may also alter serum cytokines, affecting the allergic wheal response. Additional research is necessary to determine the full potential and practical applications for clinical music interventions in dermatology. Future research should focus on targeting skin conditions that may benefit from the psychological, inflammatory, and immune effects of music.

Dermatologic toxicities of anti-neoplastic immunotherapy in United States hospitalizations.

A major side effect of anti-neoplastic immunotherapy is the onset of dermatologic toxicities following the administration of several different immunotherapy drugs. We sought to assess the incidence and mortality of anti-neoplastic immunotherapy-associated dermatologic toxicities using a nationally representative sample of United States (US) adults. The National Inpatient Sample database was queried for encounters undergoing anti-neoplastic immunotherapy. Multiple logistic regression models were constructed to analyze incidence of dermatologic toxicity and associated mortality as well as calculate adjusted odds ratios (aOR) after controlling for age, race, sex, household income, and cancer type. Immunotherapy patients demonstrated greater odds of developing pruritus (aOR = 7.59; 95% CI 5.17-11.2; P < 0.001), rash (aOR = 2.81; 95% CI 2.00-3.94, P < 0.001), generalized (aOR = 5.32; 95% CI 3.35-8.43; P < 0.001) and localized (aOR = 2.67; 95% CI 1.01-7.05; P < 0.001) skin eruptions, and vitiligo (aOR = 13.3; 95% CI 5.15-34.4; P < 0.001) compared to patients who did not receive anti-neoplastic immunotherapy. There were no significant differences observed in incidence of non-scarring alopecia (P = 0.7), psoriasis (P = 0.094), and dermatitis (P = 0.9) between the two groups. Patients who underwent immunotherapy were more likely to develop dermatologic toxicity (aOR = 3.93; 95% CI 3.12-4.94; P < 0.001), however, no significant differences in mortality were observed. Patients undergoing anti-neoplastic immunotherapy have an increased risk of developing dermatologic toxicity compared to patients who did not receive anti-neoplastic immunotherapy.

Epidemiology, management, and treatment outcomes of metastatic spinal melanoma.

Metastatic spinal melanoma is a rare and aggressive disease process with poor prognosis. We review the literature on metastatic spinal melanoma, focusing on its epidemiology, management, and treatment outcomes. Demographics of metastatic spinal melanoma are similar to those for cutaneous melanoma, and cutaneous primary tumors tend to be most common. Decompressive surgical intervention and radiotherapy have traditionally been considered mainstays of treatment, and stereotactic radiosurgery has emerged as a promising approach in the operative management of metastatic spinal melanoma. While survival outcomes for metastatic spinal melanoma remain poor, they have improved in recent years with the advent of immune checkpoint inhibition, used in conjunction with surgery and radiotherapy. New treatment options remain under investigation, especially for patients with disease refractory to immunotherapy. We additionally explore several of these promising future directions. Nevertheless, further investigation of treatment outcomes, ideally incorporating high-quality prospective data from randomized controlled trials, is needed to identify optimal management of metastatic spinal melanoma.

Clinical factors associated with skin neoplasms in individuals with Lynch syndrome in a longitudinal observational cohort.

BACKGROUND: Little is known about patient-specific risk factors for skin neoplasia in individuals with Lynch syndrome (LS). OBJECTIVE: Identify clinical factors associated with development of skin neoplasms in LS. METHODS: Clinical data were systematically collected on a cohort of LS carriers (confirmed pathogenic germline variants in MLH1, MSH2, MSH6, PMS2, or EPCAM) age ≥18 undergoing clinical genetics care at Dana-Farber Cancer Institute from January 2000 to March 2020. Multivariable logistic regression was performed to evaluate clinical factors associated with skin neoplasia. RESULTS: Of 607 LS carriers, 9.2% had LS-associated skin neoplasia and 15.0% had non-LS-associated skin neoplasia; 58.2% (353/607) had documentation of prior dermatologic evaluation; 29.7% (38/128) with skin neoplasms lacked a history of visceral LS-associated malignancy. LS-associated skin neoplasms were significantly associated with male sex, age, race, MLH1 pathogenic germline variants, MSH2/EPCAM pathogenic germline variants, and personal history of non-LS skin neoplasms. Non-LS-associated skin neoplasms was significantly associated with age, number of first- and second-degree relatives with non-LS-associated skin neoplasms, and personal history of LS-associated skin neoplasms. LIMITATIONS: Single-institution observational study; demographic homogeneity. CONCLUSIONS: Skin neoplasms are common in individuals with LS. We identified clinical factors associated with LS- and non-LS-associated skin neoplasms. Regular dermatologic surveillance should be considered for all LS carriers.

Evaluating hospital consumer-friendly price-estimator tools for shoppable services within dermatology.

A recent mandate by the Centers for Medicare and Medicaid Services (CMS) required hospitals to publish prices online. This study examined the utility of CMS-mandated price estimators for dermatology procedures. Chargemasters and price-estimator tools from the five largest hospitals in each state (n = 250) were reviewed for accessibility. Price estimators were also searched for the presence of dermatologic procedures and their estimated prices. Of the 250 hospitals included, 89.6% of chargemasters and 92.8% of price estimators were accessible. Of the available chargemasters and price estimators, 98.7% and 63.8% contained pricing data on dermatologic procedures, respectively. The most common dermatology procedures listed were debridement of subcutaneous tissue (CPT 11042) with a median price of $1,135.50 (IQR $577-$3533.67; n = 80), followed by simple incision and drainage of skin abscess (CPT 10060) with a median price of $956.00 (IQR $465.50-$6506.00, n = 79), and single punch biopsy of skin (CPT 11104) with a median price of $506.06 (IQR $356.50-$922.08, n = 29). Although price estimator tools are widely available in patient-friendly and searchable formats, they are not optimized for shoppable dermatologic services. Adding more dermatologic procedures to patient-friendly price-estimator tools can help patients directly realize savings from price shopping.