Mohs surgery for female genital Paget's disease: a prospective observational trial.

BACKGROUND: Extramammary Paget's disease recurs often after traditional surgical excision. Margin-controlled surgery improves the recurrence rate for male genital disease but is less studied for female anatomy. OBJECTIVE: This study aimed to compare surgical and oncologic outcomes of margin-controlled surgery vs traditional surgical excision for female genital Paget's disease. STUDY DESIGN: We conducted a prospective observational trial of patients with vulvar or perianal Paget's disease treated with surgical excision guided by Mohs micrographic surgery between 2018 and 2022. The multidisciplinary protocol consisted of office-based scouting biopsies and modified Mohs surgery followed by surgical excision with wound closure under general anesthesia. Modified Mohs surgery cleared peripheral disease margins using a moat technique with cytokeratin 7 staining. Medial disease margins (the clitoris, urethra, vagina, and anus) were assessed using a hybrid of Mohs surgery and intraoperative frozen sections. Surgical and oncologic outcomes were compared with the outcomes of a retrospective cohort of patients who underwent traditional surgical excision. The primary outcome was 3-year recurrence-free survival. RESULTS: Three-year recurrence-free survival was 93.3% for Mohs-guided excision (n=24; 95% confidence interval, 81.5%-100.0%) compared to 65.9% for traditional excision (n=63; 95% confidence interval, 54.2%-80.0%) (P=.04). The maximum diameter of the excisional specimen was similar between groups (median, 11.3 vs 9.5 cm; P=.17), but complex reconstructive procedures were more common with the Mohs-guided approach (66.7% vs 30.2%; P<.01). Peripheral margin clearance was universally achieved with modified Mohs surgery, but positive medial margins were noted in 9 patients. Reasons included intentional organ sparing and poor performance of intraoperative hematoxylin and eosin frozen sections without cytokeratin 7. Grade 3 or higher postoperative complications were rare (0.0% for Mohs-guided excision vs 2.4% for traditional excision; P=.99). CONCLUSION: Margin control with modified Mohs surgery significantly improved short-term recurrence-free survival after surgical excision for female genital Paget's disease. Use on medial anatomic structures (the clitoris, urethra, vagina, and anus) is challenging, and further optimization is needed for margin control in these areas. Mohs-guided surgical excision requires specialized, collaborative care and may be best accomplished at designated referral centers.

Impact of second-opinion dermatopathology reviews on surgical management of malignant neoplasms.

BACKGROUND: Second-opinion review is linked to error reduction and treatment changes in anatomic pathology. OBJECTIVE: We sought to establish the rate of diagnostic discrepancy identified by second-opinion dermatopathologic review and the effect on surgical treatment. METHODS: Cases referred for treatment of a malignant neoplasm diagnosed by an outside pathologist were reviewed. The external and internal second-opinion dermatopathologic reports were compared. Discordance in diagnosis, subtype, and treatment change owing to second-opinion review was recorded. The referring pathologist's level of dermatopathologic training was also documented. RESULTS: A total of 358 cases were included. Dermatopathologic second-opinion diagnosis was discordant with the outside diagnosis in 37 of 358 cases (10.3%). In 32 of 358 cases (8.9%), second-opinion review resulted in a change in treatment, with 28 of 32 (87.5%) of these changes resulting in cancelled surgery. Dermatologists without dermatopathologic fellowship training had the highest rate of discordant diagnoses compared with pathologists and dermatopathologists. LIMITATIONS: This was a retrospective study at a tertiary care facility. CONCLUSION: Second-opinion dermatopathologic review is associated with identification of discordant diagnoses and a substantial influence on treatment, with both cancellation of surgery and augmented management. Secondary pathologic review should be considered in high-volume surgical practices.

The utility of PRAME staining in identifying malignant transformation of melanocytic nevi.

BACKGROUND: PReferentially expressed Antigen in MElanoma (PRAME) immunohistochemical (IHC) staining is used to aid melanoma diagnosis. PRAME expression in nevus-associated melanoma (NAM) has not been evaluated. METHODS: PRAME IHC was applied to cases of NAM; staining for each population of melanocytes (benign and malignant) was graded based on the percentage of labeled cells. No labeling was graded 0, 1% to 25% labeling was 1+, 26% to 50% was 2+, 51% to 75% was 3+, and >76% was 4+. RESULTS: Thirty-six cases were reviewed. Sixty-seven percent (24/36) of melanomas were PRAME positive (4+) while no (0/36) nevi showed 4+ positivity. Eighty-one percent (29/36) of nevi were completely PRAME negative compared to 17% (6/36) of melanomas. In 67% of cases (24/36) PRAME differentiated between benign and malignant melanocyte populations. CONCLUSIONS: We identified a high rate (67%) of differential PRAME staining in adjacent benign and malignant melanocyte populations in NAM. In PRAME positive (4+) melanomas, PRAME differentiates 100% (24/24) of benign and malignant melanocyte populations. When 4+ staining is used as the threshold for positivity, PRAME staining has a sensitivity of 67% (24/36) and a specificity of 100% (36/36). These results support PRAME IHC can assist in distinguishing melanocyte populations in melanoma arising within nevi.

Comparison of psoriasis and atopic dermatitis guidelines-an argument for aggressive atopic dermatitis management.

The development of effective systemic treatments has revolutionized the treatment of inflammatory skin diseases. The availability of safe new treatments and the understanding of psoriasis as a systemic disease with comorbidities and effects on quality of life have driven the current aggressive treatment paradigm of psoriasis. Historically the morbidity of atopic dermatitis (AD) has been dismissed, given the perception of AD as "just" a rash. Differences in the guidelines for psoriasis and AD management may suggest variations in the current conceptualization of disease severity and effects on quality of life. Published guidelines from the American Academy of Dermatology for the management of psoriasis and AD were reviewed. We recorded the similarities and differences in disease assessment and therapy. The threshold to use biologic agents for moderate to severe psoriasis highlights the aggressive nature of modern psoriasis treatment. AD guidelines include an assessment of quality of life but do not designate a disease severity threshold for systemic treatment. AD and psoriasis have a tremendous effect on quality of life. The AD guidelines have a less aggressive approach to disease management than the psoriasis guidelines. We should think critically about rapid advancement to systemic agents in AD management, especially now that more and better agents are being developed.

The Role of Elimination Diets in Atopic Dermatitis-A Comprehensive Review.

BACKGROUND/OBJECTIVES: Diet is a common concern for individuals with atopic dermatitis (AD) and their families. Studies regarding the effect of dietary interventions on AD exist, but many are limited by small size and poor design. Conflicting results present a challenge to clinicians seeking to counsel patients. The aim of the current review is to examine the published literature and generate helpful conclusions for clinicians faced with dietary questions in AD. METHODS: A PubMed search was performed focusing on dietary interventions for AD in children and adults through July 2016. The search was limited to the English language and included studies that evaluated one or more forms of dietary modification for the treatment of AD. Studies of supplementation, such as with vitamins, minerals, or probiotics, were not included, nor were studies on prevention of the development of AD. A total of 43 articles met the inclusion criteria and were included in the final analysis. RESULTS: Trials varied in type, duration, and the AD patient populations studied. Overall, there is some level I evidence to support specific exclusion diets in preselected patients but insufficient evidence for strict elimination diets (diets that are typically limited to six to eight foods). Data supporting other interventions are mixed and based on small, poorly designed studies. CONCLUSIONS: A comprehensive literature review reveals some promising results and several areas in need of further study. More evidence is needed to form a strong foundation for recommendations regarding the utility and role of elimination diets in AD management, but current evidence suggests that strict diet management is not effective in the treatment AD in the vast majority of patients.

Patients' and healthcare providers' perceptions of a mobile portal application for hospitalized patients.

BACKGROUND: Hospital-based patient portals have the potential to better inform and engage patients in their care. We sought to assess patients' and healthcare providers' perceptions of a hospital-based portal and identify opportunities for design enhancements. METHODS: We developed a mobile patient portal application including information about the care team, scheduled tests and procedures, and a list of active medications. Patients were offered use of tablet computers, with the portal application, during their hospitalization. We conducted semi-structured interviews of patients and provider focus groups. Text from transcribed interviews and focus groups was independently coded by two investigators using a constant comparative approach. Codes were reviewed by a third investigator and discrepancies resolved via consensus. RESULTS: Overall, 18 patients completed semi-structured interviews and 21 providers participated in three focus groups. Patients found information provided by the portal to be useful, especially regarding team members and medications. Many patients described frequent use of games and non-clinical applications and felt the tablet helped them cope with their acute illness. Patients expressed a desire for additional detail about medications, test results, and the ability to record questions. Providers felt the portal improved patient engagement, but worried that additional features might result in a volume and complexity of information that could be overwhelming for patients. Providers also expressed concern over an enhanced portal's impact on patient-provider communication and workflow. CONCLUSIONS: Optimizing a hospital-based patient portal will require attention to type, timing and format of information provided, as well as the impact on patient-provider communication and workflow.

Cost-effectiveness analysis of margin-controlled surgery for vulvar Paget's disease.

Objectives: To determine the cost of two surgical treatment approaches for vulvar Paget's disease and model the cost-effectiveness considering differences in recurrence and reoperation over time. Methods: We assessed cost-effectiveness between excision guided by Mohs micrographic surgery (MMS-E) and traditional wide local excision (WLE). We examined billing data from patients with vulvar Paget's disease who underwent MMS-E (cases, n = 24, 2018-2022) or WLE (controls, n = 64, 1990-2020). We created typical treatment bundles incorporating physician-administered services and facility costs standardized to Medicare reimbursements in 2022 United States Dollars (USD). The primary measure of effectiveness was disease-free years of life. A secondary analysis estimated quality-adjusted life years (QALY). A Markov model simulated treatment pathways over a 10-year time horizon. Transition probabilities were based on institutional recurrence rates (3-year RR 6.7 % for MMS-E vs 34.1 % for WLE). We used a willingness-to-pay threshold of 100,000 USD per QALY. Results: The cost of a single surgical episode was 34,664 USD for MMS-E and 14,969 USD for WLE. In the setting of lower recurrence rates with MMS-E, the incremental cost was 12,789 USD per disease-free year gained. A secondary analysis incorporating QALY showed an incremental cost of 72,820 USD per QALY. Conclusions: MMS-E appears to be a cost-effective treatment for vulvar Paget's disease compared to historic standard of care. Our ability to estimate quality of life gained by avoiding disease recurrence was limited by scant data for this rare condition; thus, future studies incorporating health utility values are needed to facilitate a more comprehensive analysis.

Dermatologic surgeons' approaches to acral lentiginous melanoma: a survey of the American College of Mohs Surgery.

Acral lentiginous melanoma (ALM) is a subtype of melanoma that primarily affects ethnic minorities and is associated with poor outcomes. Racial inequalities in melanoma survival, particularly between Blacks and Whites, have been well documented in the literature. There are limited data on the role of surgical approach to ALM and its contribution to the existing disparities in ALM survival. Thus, this study aimed to investigate the role of surgical approach to ALM among Mohs surgeons. The survey was distributed to members of the American College of Mohs Surgery (ACMS), with 43 physicians completing the survey. The results showed that there was no consensus among Mohs surgeons regarding the surgical approach for ALM, even for advanced stages. 74% respondents performed complete circumferential peripheral and deep margin assessment (CCPDMA) for ALM, including Mohs micrographic surgery (MMS) or en face staged excision. However, barriers such as limited training, low comfort operating on acral sites, and inadequate access to specialized biopsies were reported. Treatment recommendations varied widely regardless of the ALM stage, with no significant differences based on exposure to ALM during training, practice setting, or percent of skin of color patients served. These findings highlight the lack of guidelines and the need for more data on outcomes to support optimal management of ALM. The study emphasizes the importance of addressing healthcare disparities and improving survival outcomes, particularly for ethnic minorities affected by ALM. Consensus guidelines could help standardize treatment approaches and potentially reduce disparities in ALM management.

An Evidence-Based Approach to Pediatric Melanonychia.

Melanonychia including melanonychia striata in children poses a diagnostic dilemma. Atypical clinical features often raise the possibility of malignancy, and a nail unit biopsy may be recommended. Commensurate with atypical clinical features, the histopathology may also appear alarming. However, accumulating data illustrate that most cases of melanonychia striata are benign and suggest that an alternate approach is often warranted for pediatric patients. Herein, we review the existing data regarding pediatric melanonychia striata and offer an evidence-based approach to its evaluation and management.

Device Safety Implications of the Clinical Data Leading to US Food and Drug Administration Approval of Soft-Tissue Fillers: A Systematic Review.

IMPORTANCE: The US Food and Drug Administration (FDA) recently issued a safety warning regarding soft-tissue fillers (STFs) based on the risk of blindness and facial necrosis. OBJECTIVE: To examine the quality of evidence leading to FDA approval of STFs. EVIDENCE REVIEW: All original approvals for STFs were mined using the publicly available FDA database. The Cochrane Collaboration's risk of bias assessment tool was applied to all randomized clinical trials (RCTs). FINDINGS: A total of 14 STF approvals were identified. Of those, 10 pivotal studies (71%) were RCTs and 9 (60%) were masked. The median number of patients per trial was 144 (range, 30-439). Eleven of 12 studies (92%) met their primary end point. Ten of 14 trials (71%) involved injections solely of the nasolabial folds or cheeks; only 4 trials involved treatment of other facial regions. All 10 RCTs had an unclear risk of selection bias. Only 2 RCTs reported exclusions and attrition. CONCLUSIONS AND RELEVANCE: Safety warnings relate more to the off-label use of STFs, which has not been sufficiently studied prospectively. Although STFs remain a safe device, with approval based mostly on RCT outcomes, implementation of unique device identifiers and greater use of physician-led registries would ensure physician, consumer, and regulatory confidence in STF safety.

Consumer Preferences, Product Characteristics, and Potentially Allergenic Ingredients in Best-selling Moisturizers.

Importance: Because moisturizer use is critical for the prevention and treatment of numerous dermatological conditions, patients frequently request product recommendations from dermatologists. Objective: To determine the product performance characteristics and ingredients of best-selling moisturizers. Design and Setting: This cohort study involved publicly available data of the top 100 best-selling whole-body moisturizing products at 3 major online retailers (Amazon, Target, and Walmart). Products marketed for use on a specific body part (eg, face, hands, eyelids) were excluded. Main Outcomes and Measures: Pairwise comparisons of median price per ounce on the basis of marketing claims (eg, dermatologist recommended, fragrance free, hypoallergenic) and presence of ingredients represented in the North American Contact Dermatitis Group (NACDG) series were conducted using Wilcoxon rank sum tests. The effect of vehicle type (eg, ointment, lotion, cream, butter) was assessed using the Kruskal-Wallis test. Cross-reactors and botanicals for fragrances were derived from the American Contact Dermatitis Society's Contact Allergen Management Program database. Results: A total of 174 unique best-selling moisturizer products were identified, constituting 109 713 reviews as of August 2016. The median price per ounce was $0.59 (range, $0.10-$9.51 per ounce) with a wide range (9400%). The most popular vehicles were lotions (102 [59%]), followed by creams (22 [13%]), oils (21 [12%]), butters (14 [8%]), and ointments (3 [2%]). Only 12% (n = 21) of best-selling moisturizer products were free of NACDG allergens. The 3 most common allergens were fragrance mix (n = 87), paraben mix (n = 75), and tocopherol (n = 74). Products with the claim "dermatologist recommended" had higher median price per ounce ($0.79; interquartile range [IQR], $0.56-$1.27) than products without the claim ($0.59; IQR, $0.34-$0.92). Products with the claim "phthalate free" had higher median price per ounce ($1.38; IQR, $0.86-$1.63) than products without the claim ($0.59; IQR, $0.35-$0.91). Lotions (median, $0.49; IQR, $0.31-0.68) were statistically less expensive per ounce than butters (median, $1.20; IQR, $0.76-$1.63), creams (median, $0.80; IQR, $0.69-$1.25) and oils (median, $1.30; IQR, $0.64-$2.43). For products with a claim of "fragrance free," 18 (45%) had at least 1 fragrance cross-reactor or botanical ingredient. Products without any ingredients in the NACDG (median, $0.83; IQR, $0.47-$1.69) were not statistically more expensive per ounce than products with 1 or more allergens (median, $0.60; IQR, $0.35-$1.06). Conclusions and Relevance: Best-selling moisturizers vary widely by price and product characteristics. Given the lack of readily available comparison data on moisturizer efficacy, dermatologists should balance consumer preference, price, and allergenicity in their recommendations.