Dermatology最新文献

Introduction: Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition that disproportionately affects women and skin of color. In this single-center study, we investigate the epidemiologic landscape of HS individuals in South Florida, which possesses a large and diverse Hispanic population.

Methods: A retrospective chart review from the University of Miami Hospitals and Clinics was conducted, identifying HS patients between 2010 and 2023 according to the pertinent International Classification of Diseases (ICD) codes. Data extraction included demographic information, past medical history, and laboratory results. Chi-squared tests and logistic regression analyses were performed to detect statistically significant relationships.

Results: Among the 3,515 identified charts, almost half were identified as Hispanic or Latino (47.0%), and 31.7% identified as Black or African American (AA). Over 50% of patients had elevated haptoglobin and C-reactive protein (CRP) levels (51.2% and 53.4%, respectively). There were also trends of hyperuricemia in HS patients, though this did not reach statistical significance. Female sex was associated with a higher prevalence of pilonidal disease (PD) (odds ratio [OR] 2.99, p < 0.001), particularly in Hispanic females compared to non-Hispanic females (OR 3.22, p < 0.001, and OR 2.74, p < 0.001, respectively).

Conclusion: In South Florida, HS disproportionately affects AA and Hispanic/Latino women. Obesity and metabolic syndrome are commonly associated with HS, and uric acid may serve as a potential marker for metabolic derangement, although larger studies are needed to investigate this relationship. Female patients with HS are at increased risk of developing PD, highlighting the need for personalized comorbidity screening and therapeutic management of HS.

Introduction: The coexistence of multiple immune-mediated inflammatory diseases (IMIDs) in a single patient represents a growing clinical challenge, yet comprehensive data on patients with more than two IMIDs remain limited. The aim of this study was to characterize the clinical profiles, therapeutic responses, and quality of life outcomes in patients presenting with complex IMID associations involving cutaneous manifestations.

Methods: We established the CIMID-Skin registry, a prospective observational study enrolling adult patients with multiple IMIDs including at least one dermatological condition. Patients were stratified into control (2 IMIDs) and complex (>2 IMIDs) groups. Clinical data validated severity scores, and patient-reported outcomes were systematically collected.

Results: The cohort (n = 54) was predominantly female (77.8%), with a mean age of 45.4 years and overweight range body mass index (27.4 kg/m2). Patients frequently presented with multiple IMIDs, with 59.3% having ≥3 conditions. The most common diseases were psoriasis and hidradenitis suppurativa (42.6% each) and psoriatic arthritis (40.7%). Paradoxical reactions were observed in a significant proportion of patients, with paradoxical psoriasis reported in 24.1% of the cohort. Quality of life was markedly impaired, with 65.2% showing moderate-to-extreme DLQI impact, and 24.1% reaching the WHO-5 depression threshold.

Conclusions: Patients with complex IMID associations demonstrate distinct clinical patterns and therapeutic challenges. The high prevalence of paradoxical reactions and the specific clustering of certain IMIDs suggest shared pathophysiological mechanisms that warrant further investigation.

Introduction: Pemphigus is a rare group of autoimmune blistering diseases, most often mediated by IgG autoantibodies but occasionally involving IgA or other immunoglobulin classes, characterized by the loss of epidermal cell adhesion due to desmoglein-targeted autoantibodies. Rituximab, an anti-CD20 monoclonal antibody, has emerged as a novel and effective treatment, offering targeted B-cell depletion.

Methods: A prospective observational study was conducted on 51 patients with confirmed pemphigus. Patients received either the RA protocol (1 g rituximab 2 weeks apart) or the lymphoma protocol (375 mg/m2 weekly for 4 weeks). Disease stages were defined according to the international consensus on pemphigus [Murrell et al., J Am Acad Dermatol. 2008;58(6):1043-6]. Desmoglein 1 and 3 levels were measured using ELISA at baseline and at 6 months. Patients receiving DCP therapy were analyzed separately but excluded from primary rituximab remission analysis. Clinical remission, relapse, and adverse events were recorded.

Results: Most patients (70.59%) were diagnosed with pemphigus vulgaris, and 72.55% achieved complete remission. Mean Dsg1 levels significantly reduced from 211.96 ± 109.10 to 10.95 ± 25.43 and Dsg3 from 206.08 ± 105.11 to 17.69 ± 47.79 (p < 0.001). Patients receiving the RA protocol demonstrated a slightly greater reduction in Dsg3 titers compared to the lymphoma group, though not statistically significant. Recalcitrant disease was noted in 21.57% of cases. The low RA protocol was the most commonly used (70.59%). Adverse events were minimal and included mild infusion reactions. Mortality was reported in 5.88% of cases.

Conclusion: Rituximab therapy demonstrates robust clinical and immunological efficacy in pemphigus, with substantial reductions in Dsg1 and Dsg3 antibody levels and high remission rates. Its use, particularly with the low RA protocol, offers a safer and more effective alternative to conventional immunosuppressive regimens. Patients on DCP therapy had slower antibody decline and greater treatment toxicity compared to rituximab, reaffirming rituximab's superiority.

Introduction: Artificial intelligence (AI) can potentially assist in triaging suspicious skin lesions as malignant or benign. General-purpose multimodal large language models (LLMs), such as GPT-4o, have not been rigorously evaluated for this task. This study assessed GPT-4o's ability to triage skin lesions and compared its performance to specialised neural networks.

Methods: We evaluated GPT-4o using 1,000 random cases from the PAD-UFES-20 dataset with 50 repeated trials. GPT-4o was tested using clinical data-only, image-only, and multimodal inputs. GPT-4o's performance, consistency, and fairness across different demographic subgroups was evaluated. Its performance metrics were compared against specialised unimodal and multimodal neural networks trained on a separate subset of the PAD-UFES-20 dataset.

Results: GPT-4o exhibited poor triage performance across all modalities, with average balanced accuracies of 0.571, 0.602, and 0.622 for clinical data, image, and multimodal inputs, respectively. Sensitivity was consistently high (>0.95) with the trade-off of very low specificity. Mean agreement rates were high (>0.90); however, Fleiss' κ indicated only moderate consistency due to a strong bias toward malignant classifications. Fairness evaluations showed poorer discriminative performance in younger patients compared to middle-aged and elderly patients but no notable differences between different sex and skin tone subgroups. Specialised neural networks significantly outperformed GPT-4o on most pairwise comparisons. Multimodal inputs significantly improved GPT-4o performance over unimodal inputs.

Conclusion: Although GPT-4o consistently triaged skin lesions with high sensitivity, its very low specificity limits clinical utility. Thus, general-purpose LLMs like GPT-4o are currently unsuitable for clinical dermatological diagnostics without significant field-specific developments and validation.

Introduction: The differences in all-cause and cause-specific mortality among patients with generalized pustular psoriasis (GPP), palmoplantar pustulosis (PPP), and psoriasis vulgaris (PV) are not well understood. The aim of our study was to compare all-cause and cause-specific mortality in patients with GPP, PPP, and PV.

Methods: We conducted a nationwide, retrospective cohort study using data from the Korean National Health Insurance Service and National Death Registry between 2002 and 2022. Patients with GPP, PPP, and PV aged ≥18 years were identified based on ≥3 visits with principal diagnosis codes. Baseline characteristics, including demographics, general health data, socioeconomic status, and comorbidity profiles, were balanced using inverse probability weighting. Participants were followed up from the index date until the occurrence of death, emigration, or the end of the observation period to investigate all-cause and cause-specific mortality.

Results: Multivariate regression analysis showed that patients with GPP had significantly higher all-cause mortality than those with PV (adjusted hazard ratio [aHR], 1.23; 95% confidence interval [CI], 1.20-1.25), while patients with PPP had lower all-cause mortality (aHR, 0.89; 95% CI, 0.87-0.91). GPP was associated with higher cause-specific mortality, particularly from hematologic (aHR, 1.76; 95% CI, 1.27-2.44) and respiratory diseases (aHR, 1.37; 95% CI, 1.30-1.46), whereas PPP patients had lower mortality from gastrointestinal (aHR, 0.57; 95% CI, 0.52-0.63) and infectious diseases (aHR, 0.71; 95% CI, 0.63-0.80). Subgroup and sensitivity analyses confirmed these findings.

Conclusions: GPP is associated with higher all-cause and cause-specific mortality than PV, while PPP is linked to lower mortality, indicating potential epidemiological and biological differences among these subtypes.

Introduction: Children with atopic dermatitis (AD) appear to have higher skin permeation, which may increase the risk of systemic adverse events following administration of potent topical corticosteroids, such as clobetasol propionate (CP). However, the assessment of dermal absorption in a controlled clinical trial in children is not feasible. Using model-based approaches, this study aimed to characterise stratum corneum (SC) and systemic exposure to CP following topical application of the cream formulation and to investigate its effects on circulating cortisol levels and growth velocity (GV) in children with AD.

Methods: Physiologically based pharmacokinetic and pharmacokinetic-pharmacodynamic modelling were utilised to describe the dermal absorption of CP in a virtual cohort of paediatric patients (1 to <18 years old). Based on a skin impairment model for lesional and non-lesional skin, CP concentrations in the SC and in plasma were described over time. Simulation scenarios were then implemented to evaluate the effect of varying treatment conditions on the systemic exposure to CP, circulating cortisol levels, and GV. Subsequently, clinical cases were simulated to illustrate typical cases of AD in the paediatric population.

Results: Surface area had the largest impact on local and systemic concentrations of CP, while body site and age had a minor effect. When comparing similar surface areas and site of application, the dose, dosing regimen (o.d. vs. b.i.d.), and occlusion had no clinically relevant effect on local and systemic exposure. Assuming a uniform application (1.2 mg/cm2), CP cream can be applied on up to 20% of the body surface area in children with AD over a period of 2 weeks. The short-term use of CP cream had no effect on the GV of children with AD.

Conclusion: Surface area affected by AD should be considered when assessing the risk of systemic side effects. CP can be applied up to 20% of the body surface area of a child (>1-year-old) without clinically relevant changes to circulating cortisol levels. In contrast to the systemic effects of oral corticosteroids, the short-term use of CP has no impact the GV of paediatric subjects with AD.

Introduction: Generalized pustular psoriasis (GPP) and pyoderma gangrenosum (PG) are rare, severe neutrophilic dermatoses with overlapping autoinflammatory pathways. Although case reports suggest a link between GPP and PG, no population-based studies have quantified this risk.

Methods: We performed a retrospective cohort study using the TriNetX federated electronic health record network. Adults (≥18 years) with ICD-10 codes for GPP (L40.1) or non-GPP (L40.0-L40.9 excluding L40.1) from 2018 to 2023 were identified. Controls had no psoriasis diagnosis. Cohorts were 1:1 propensity score matched for age, sex, race, ulcerative colitis, Crohn's disease, systemic lupus erythematosus, and rheumatoid arthritis. The primary outcome was new PG diagnosis (L88) post-index. Hazard ratios (HRs) for PG were estimated via matched survival analysis.

Results: After matching, the GPP cohort (n = 44,187) had a significantly higher risk of PG than controls (n = 44,187; HR 5.14; 95% CI 2.77-9.53). Non-GPP patients (n = 393,833) also showed elevated PG risk versus controls (n = 393,833; HR 3.83; 95% CI 3.00-4.90). In direct comparison (n = 193,208 each), GPP had greater PG risk than non-GPP (HR 2.33; 95% CI 1.46-3.71). Median follow-up ranged from 687 to 886 days across comparisons.

Conclusion: This first population-based evidence demonstrates that GPP confers a markedly increased risk of PG compared to both controls and other psoriasis subtypes. These findings underscore the need for heightened clinical vigilance for PG in GPP patients and may inform targeted surveillance strategies.

Introduction: Autoimmune blistering diseases (AIBDs) are severe, life-threatening conditions known for their debilitating physical and psychological impacts. The conditions also have a high rate of recurrence, leading to patient distress. This study adapted and validated a widely used measure, the Fear of Cancer Recurrence Inventory - Short Form, for use with patients with AIBDs.

Methods: Adult volunteers (n = 219) with AIBD completed an anonymous online questionnaire battery. In addition to the Fear of Blistering Disease Recurrence Inventory - Short Form (FBDRI-SF), participants provided demographic and disease course information and completed the ultra-brief Patient Health Questionnaire for Depression and Anxiety (PHQ-4).

Results: The FBDRI-SF demonstrated excellent psychometric properties. Scores on the FBDRI-SF were associated with disease course, depression, and anxiety, suggesting that it is a valid indicator of clinically significant psychological distress.

Conclusion: The FBDRI-SF is a brief and useful screen to identify AIBD patients likely to benefit from psychological evaluation and support.

Introduction: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful, rapidly progressive ulcerations, often associated with systemic inflammatory diseases. Despite advancements in diagnostic criteria, PG remains a diagnostic challenge, and recent increases in reported cases may reflect improved recognition rather than true incidence. This study evaluates epidemiological trends in PG diagnosis in Italy (2013-2024) across four dermatology centers (Bologna, Milano, Pisa, Torino) to determine whether increased number of diagnoses are due to refined diagnostic methodologies or external immunological factors.

Methods: A cross-sectional analysis was conducted using anonymized data from four major dermatology centers in Italy. Data analysis deployed one-way ANOVA and Poisson regression models to assess temporal trends in PG diagnoses. Significance was set at α = 0.05.

Results: Between 2013 and 2024, 213 PG cases were diagnosed across four Italian referral centers, with a female predominance (126 vs. 87). Diagnostic rates showed marked annual variability, including a 150% rise in 2015, a 68.8% drop in 2020, and a 360% rebound in 2021. Diagnoses more than doubled after 2018 (68 vs. 145; p < 0.05). Trends varied by center: Bologna (+0.25/year, p = 0.003) and Milano (+0.09/year, p = 0.03) showed significant increases; Pisa and Torino did not. Overall, the rise in diagnoses post-2018 aligns with the broader adoption of standardized diagnostic criteria.

Conclusion: The increase in PG diagnoses recorded since 2018 is more plausibly explained by improved clinical recognition and widespread adoption of structured diagnostic frameworks than by a true rise in incidence. Regional variability and limitations of retrospective data caution against firm epidemiological conclusions. Prospective multicenter studies and standardized registries are needed to validate diagnostic tools, reduce misclassification, and clarify the true burden of PG.