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Background: Previous case-control studies have suggested that environmental factors including exposure to pesticides and organic materials, diet and medications have an important role in the pathogenesis of pemphigus vulgaris. These studies lacked geographical population controls and had less than three controls per case., Objective: To identify environmental and occupational risk factors associated with the development of pemphigus vulgaris (PV) and bullous pemphigoid (BP)., Method: Cases were patients with PV ( n = 25) and BP ( n = 29) recruited from 2009 to 2017. Controls for PV ( n = 72) and BP ( n = 84) were recruited from the general population via electoral commission matching, matched for age, sex, residential location, and ethnicity. Data about demographics, environmental exposures and occupational exposures, was collected using a structured questionnaire. Conditional logistic regression analysis was undertaken using SPSS software to identify significant variables., Results: Significant factors associated with PV included the daily consumption of leeks (odds ratio (OR) 3.6; p = 0.025), mustard oil (OR = 4.4; p = 0.049), tomatoes (OR = 4.735; p = 0.032), multivitamins (OR 3.6; p = 0.009), alcohol (0.039), and calcium supplements (OR = 44, p < 0.001). Other associated factors included the number of lifetime sunburns ( p = 0.019), high levels of mental stress ( p < 0.001), and the use of lime household cleaning products ( p < 0.001), Significant factors associated with BP included the daily consumption of green or herbal tea (OR = 3.7; p = 0.004), fish oil (OR = 5.7; p < 0.001), calcium supplements (OR = 6.1; p < 0.001), multivitamins (OR = 2.6; p = 0.043), and glucosamine (OR = 3.0; p = 0.046). The use of lime household cleaning products ( p < 0.001) and high levels of mental stress ( p = 0.007) were also associated with BP., Conclusion: Dietary factors containing thiol groups such as leeks, tomatoes, and mustard oil may be potential triggers for PV. High levels of mental stress, the use of supplementary medications such as calcium and multivitamins, and chemical cleaning products containing lime may be associated with an increased risk of developing both PV and BP. Lifestyle changes should be part of routine management for these patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Stone, Bak, Oh, Zhao, Venugopal, Kumar and Murrell.)

Background: Pemphigus is a rare autoimmune blistering disease with potentially life-threatening consequences. Establishing minimal clinically important differences (MCIDs) for disease severity scores like the Pemphigus Disease Area Index (PDAI) is crucial for assessing treatment efficacy., Objectives: To calculate MCIDs for both improvement and deterioration in PDAI scores in patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF), using the anchor-based method., Methods: A total of 41 patients with pemphigus were recruited, with 35 meeting the MCID analysis criteria. The anchor-based method was used to calculate MCIDs for PDAI scores against the 15-point Likert scale and the Physician Global Assessment visual analogue scale (PGA-VAS) anchors. Receiver operating characteristic curves were employed to determine optimal MCID cutpoints with the highest Youden Index (J). The 15-point Likert scale scores the change in disease severity spanning from -7 to +7, designed to quantify the extent of disease improvement/deterioration since the preceding visit., Results: The MCID for improvement in PDAI activity scores was 2.65 points using the 15-point Likert scale (78.7% correct classification; sensitivity 75.9%; specificity 73.5%) and 2.5 points using the PGA-VAS as the anchor (78.0% correct classification; sensitivity 84.4%; specificity 68.2%). Given the slightly higher correct classification rate using the 15-point Likert scale anchor, the MCID of 2.65 points was selected for PDAI activity score improvement. In contrast, the MCID for deterioration consistently remained at 2.5 points for the 15-point Likert scale anchor (81.0% correct classification; sensitivity 72.7%; specificity 81.0%) and 2.5 points for the PGA-VAS anchor (70.9% correct classification; sensitivity 69.6%; specificity 76.9%)., Conclusions: This study marks the inaugural attempt at MCID determination for PDAI scores in pemphigus, filling a critical knowledge gap. The study's calculated MCIDs provide essential benchmarks for clinical trials, treatment evaluation and research design optimization. Future studies should explore international collaborations, to examine potential cross-cultural variations in MCIDs., Competing Interests: Conflicts of interest D.F.M. was a co-investigator/co-creator of the Pemphigus Disease Area Index. The other authors declare no conflicts of interest., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists.)

Bullous pemphigoid (BP) is an autoimmune blistering disease that can have a profound negative impact on quality of life. BP most often affects the elderly, a population with a high medical burden and special safety concerns. In this review, we outline the BP disease course, diagnosis, epidemiology and comorbidities, and describe tools commonly used to assess BP disease activity and severity and the impact of BP on health-related quality of life. We also outline biologic treatments currently under investigation for the treatment of BP and highlight the importance of considering safety when treating elderly patients., (© 2024 Regeneron Pharmaceuticals. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.)

This review aims to present a comprehensive synthesis of the existing treatment modalities for keratosis pilaris (KP) and evaluate their therapeutic efficacy. KP is a prevalent chronic dermatological condition typified by its unique 'chicken skin appearance', with the cheeks being the most commonly involved sites. Numerous therapeutic interventions have emerged, given its substantial prevalence and impact on skin aesthetics and psychological wellbeing. Nonetheless, a consistent therapeutic response has been challenging to achieve. This review endeavours to collate and critically appraise the current treatment landscape for KP. An exhaustive literature search was performed using databases such as Ovid, PubMed and Scopus. From an initial count of 459 articles identified after deduplication, 52 were selected for inclusion after a thorough full-text examination for articles with concrete outcome data highlighting the efficacies of different therapeutic modalities; articles that lacked data or were tangential to the core focus on KP treatment were excluded. The included articles were then catalogued based on the nature of treatment strategies and their respective outcomes. Among the various therapeutic interventions, laser and light modalities appear to be supported by the most substantial evidence base. Notably, the Nd:YAG (neodymium-doped yttrium-aluminium-garnet) laser, attributed to its longer wavelength, emerged as a preferred option. While other therapeutic avenues have also exhibited notable improvements in skin texture and discolouration relative to baseline, the inconsistency in outcome measures underscores the need for a standardized, KP-specific scoring system to foster a more coherent comparison across treatments. Based on the current evidence, Nd:YAG laser therapy demonstrates promising effectiveness with a relatively favourable side-effect profile. However, the landscape of KP treatment is multifaceted, and further studies are essential to solidify recommendations., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Background: Janus kinase inhibitors (JAKinibs) have the potential to dramatically alter the landscape of atopic dermatitis (AD) management due to their promising efficacy results from phase 3 trials and rapid onset of action. However, JAKinibs are not without risk, and their use is not appropriate for all AD patients, making this a medication class that dermatologists should understand and consider when treating patients with moderate-to-severe AD., Objective: This consensus expert opinion statement from the International Eczema Council (IEC) provides a pragmatic approach to prescribing JAKinibs, including choosing appropriate patients, dosing, clinical and lab monitoring, as well as long-term use., Methods: An international cohort of authors from the IEC with expertise in JAKinibs selected topics of interest and were formed into authorship groups covering 10 subsections. The groups performed topic-specific literature reviews, consulted up-to-date adverse event (AE) data, referred to product labels and provided analysis and expert opinion. The manuscript guidance and recommendations were reviewed by all authors as well as the IEC Research Committee., Results: We recommend JAKinibs be considered for patients with moderate to severe AD seeking the benefits of rapid reduction in disease burden and itch, oral administration, and the potential for flexible dosing. Baseline risk factors should be assessed prior to prescribing JAKinibs, including increasing age, venous thromboembolisms, malignancy, cardiovascular health, kidney/liver function, pregnancy and lactation, and immunocompetence. Patients being considered for JAKinib therapy should be current on vaccinations and we provide a generalized framework for laboratory monitoring, though clinicians should consult individual product labels for recommendations as there are variations among the JAKinib class. Patients who achieve disease control should be maintained on the lowest possible dose, as many of the observed AEs occurred in a dose-dependent manner. Future studies are needed in AD patients to assess the durability and safety of continuous long-term use of JAKinibs, combination medication regimens, and the effects of flexible, episodic treatment over time., Conclusions: The decision to initiate a JAKinib should be shared among patient and provider, accounting for AD severity and personal risk/benefit assessment, including consideration of baseline health risk factors, monitoring requirements and treatment costs., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists.)

Objectives: To describe disease burden in individuals with moderate-to-severe atopic dermatitis (AD) in Australia and New Zealand (ANZ) and compare it with other geographic regions., Methods: This multicentre, cross-sectional, observational study (MEASURE-AD) recruited consecutive adolescent and adult patients attending dermatology clinics in 28 countries. Data collected included scores of pruritus, disease severity, sleep, pain, disease control, work and quality of life., Results: This study included 112 ANZ participants (Australia n = 72; New Zealand n = 40) from December 2019 to December 2020. Treatments included topicals (85.7% of patients), non-biologic systemic therapy (28.6%), phototherapy (9.8%) and dupilumab (4.5%). Mean Eczema Area and Severity Index (EASI) score was 22.3 (95% CI 19.6-25.0) and Patient-Oriented Eczema Measurement (POEM) score was 18.4 (95% CI 16.8-20.0). Pruritus Numerical Rating Scale (NRS) was 6.0 (95% CI 5.5-6.6) (50% had severe pruritus) and Dermatology Life Quality Index (DLQI) 14.3 (95% CI 12.8-15.8). ADerm-Impact sleep domain score was 15.1 (95% CI 13.2-16.9). ADerm-Symptom Scale worst skin pain domain score was 5.0 (95% CI 4.3-5.6). Work Productivity and Activity Impairment (WPAI) percentages indicated work and productivity impairment. Inadequately controlled AD was self-reported by 41%, with 9.7 flares in the past 6 months. Scores of pruritus, disease severity, sleep, pain, disease control and quality of life in ANZ were often the highest of all the geographic regions studied., Conclusion: ANZ patients with AD have a high disease burden, which extends across multiple facets of daily life. Many are inadequately controlled with existing therapies., (© 2024 Australasian College of Dermatologists.)

Digital twins, innovative virtual models synthesizing real-time biological, environmental, and lifestyle data, herald a new era in personalized medicine, particularly dermatology. These models, integrating medical-purpose Internet of Things (IoT) devices, deep and digital phenotyping, and advanced artificial intelligence (AI), offer unprecedented precision in simulating real-world physical conditions and health outcomes. Originating in aerospace and manufacturing for system behavior prediction, their application in healthcare signifies a paradigm shift towards patient-specific care pathways. In dermatology, digital twins promise enhanced diagnostic accuracy, optimized treatment plans, and improved patient monitoring by accommodating the unique complexities of skin conditions. However, a comprehensive review across PubMed, Embase, Web of Science, Cochrane, and Scopus until February 5th, 2024, underscores a significant research gap; no direct studies on digital twins' application in dermatology is identified. This gap signals challenges, including the intricate nature of skin diseases, ethical and privacy concerns, and the necessity for specialized algorithms. Overcoming these barriers through interdisciplinary efforts and focused research is essential for realizing digital twins' potential in dermatology. This study advocates for a proactive exploration of digital twins, emphasizing the need for a tailored approach to dermatological care that is as personalized as the patients themselves., (© 2024. Crown.)

Background: Scoring systems play a crucial role in dermatology by providing objective measurements of disease severity, treatment efficacy, and outcome comparisons. In autoimmune blistering diseases (AIBDs), standardized scoring systems are essential for accurate evaluations; however, there is currently a lack of consensus on scoring methods., Objective: This literature review explores scoring systems in AIBDs by tracing their development, addressing challenges, and highlighting their role in defining endpoints, regulatory considerations, and clinical trials., Materials and Methods: Existing scoring systems for AIBDs, such as the Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, Pemphigus Oral Lesions Intensity Score, Oral Disease Severity Score, and Pemphigus Vulgaris Activity Score, are examined for their validity, reliability, and responsiveness. The Bullous Pemphigoid Disease Area Index for bullous pemphigoid is also discussed. The concept of minimal clinically important differences is explored to determine clinically significant improvements in disease severity., Conclusion: This review provides a comprehensive understanding of the central role of scoring systems in dermatology and their implications for research and clinical practice in AIBDs., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Indian Dermatology Online Journal.)

Competing Interests: There are no conflicts of interest.

Efforts to achieve gender equity of health professionals should be a priority in all fields of medicine, including academic dermatology. This review aimed, first, to summarize available evidence about the status of gender equity in various domains of academic dermatology-headship positions, salary, editor and editorial board appointments, publications, conference presentations, receipt of research grants and academic prizes-second, to identify challenges to achieving gender equity and, third, to articulate the components of a multifaceted strategy for gender parity. A variety of databases were searched. Manual searching of reference lists and searching of grey literature were also undertaken. It was found that, despite improvements in some domains, the gender inequity persists in all of the above-mentioned areas of academic dermatology. Challenges to achieve gender parity include time in pregnancy, disproportionate participation in childrearing and domestic tasks compared with men, suboptimal legislation in many jurisdictions for parenting and childcare leave, and unconscious biases about women. Elements of a multipronged approach include strengthening women's dermatology societies that advocate for women in academia; celebrating the careers of distinguished female academic dermatologists; mentoring; promoting leadership courses; striving for a greater representation of women among editors-in-chief, authors, and conference presenters, among others; seeking better pay, leave conditions and other work entitlements; conducting high-quality research about gender inequity in academic dermatology; imposing sanctions for violations of gender equity; supporting dermatologists' health; and learning from the experience of other fields of academic medicine., (© 2024 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Introduction: The review article explores the evolving role of Bruton's tyrosine kinase (BTK) inhibitors in immune-mediated dermatological conditions, addressing significant gaps in current treatment approaches., Areas Covered: The review comprehensively discusses the mechanisms of action of BTK inhibitors, including irreversible and reversible inhibitors. Clinical applications of BTK inhibitors in dermatological diseases such as pemphigus, chronic spontaneous urticaria (CSU), hidradenitis suppurativa (HS), systemic lupus erythematosus (SLE), and atopic dermatitis are explored, highlighting recent advancements and ongoing clinical trials. Potential advantages of BTK inhibitors over existing therapies and challenges in translating preclinical findings to clinical outcomes are discussed., Expert Opinion/commentary: BTK inhibitors represent a promising therapeutic avenue for immune-mediated dermatological conditions, offering oral administration, targeted pathway inhibition, and a favorable safety profile compared to biologic therapies. Ongoing research and clinical trials hold the potential to address unmet needs and reshape the therapeutic landscape in dermatology.

Trial Design: Pemphigus is a rare but life-threatening autoimmune disease requiring long-term treatment that minimizes corticosteroid (CS) exposure while providing consistent disease control. The phase 2 pemphigus study of oral, reversible, covalent Bruton tyrosine kinase inhibitor rilzabrutinib demonstrated rapid and sustained efficacy with well-tolerated safety., Methods: Adults (aged 18-80 years) were randomized 1:1 to 400 mg rilzabrutinib (n = 65) or placebo (n = 66) twice daily (with CS ≤ 0.5 mg/kg/d) for 37 weeks in the phase 3 PEGASUS study in moderate-to-severe pemphigus vulgaris/pemphigus foliaceus., Results: The primary endpoint of complete remission from week 29 to week 37 with the amended endpoint CS dose ≤10 mg/d was not significant for 13 of 54 (24%) rilzabrutinib versus 10 of 55 (18%) placebo patients with PV (P = .45). Secondary endpoints showed numerical but nonsignificant improvements with rilzabrutinib (vs placebo) in reduced CS use, prolonged complete remission duration, and faster time to first complete remission., Conclusions: Overall, rilzabrutinib was well-tolerated, with similar adverse events reported in both groups. Using minimal CS dose ≤10 mg/d and excluding remote observations, the primary efficacy endpoint was not met. However, results from a prespecified sensitivity analysis using CS dose ≤5 mg/d, considering all observations, and including all patients support Bruton tyrosine kinase inhibition as a viable therapeutic approach for pemphigus., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Bullous pemphigoid (BP) is a rare, autoimmune, blistering skin disease associated with high disease burden, profoundly decreased quality of life and increased morbidity. Emerging evidence supports an important role for type 2 inflammation in disease pathogenesis. Current management relies on topical and/or systemic corticosteroids, non-selective immunosuppressants and antibiotics with anti-inflammatory properties, which are all limited by side effects and toxicities. Therefore, targeted, efficacious and safe therapies are needed. Dupilumab blocks the shared receptor component for interleukin (IL)-4 and IL-13, key and central drivers of type 2 inflammation. Several reports of patients successfully treated with dupilumab have been published; however, dupilumab has not been formally assessed in a double-blind, placebo-controlled trial., Objectives: We report the design of LIBERTY-BP ADEPT, a multicenter, randomized, double-blind, placebo-controlled clinical trial to evaluate the efficacy and safety of dupilumab in adults with BP., Methods: LIBERTY-BP ADEPT comprises a 35-day screening, 52-week treatment and 12-week follow-up period. Approximately 98 adults aged 18-90 years with moderate-to-severe BP are being enrolled at 51 sites on 4 continents and randomized 1:1 to subcutaneous dupilumab or placebo every 2 weeks. All participants will receive concomitant oral corticosteroids (OCS)., Planned Outcomes: The primary endpoint is the proportion of patients achieving complete remission off steroid therapy at week 36. Key secondary endpoints include total cumulative OCS dose to week 36, percent change and proportion of patients with ≥ 4-point reduction in the weekly average of daily Peak Pruritus Numerical Rating Scale from baseline to week 36 and percent change in Bullous Pemphigoid Area Index score from baseline to week 36., Conclusion: The trial results will provide evidence on whether the efficacy and safety of dupilumab support its use as a potential novel treatment approach for BP and will provide new insights into the role of type 2 inflammation in BP pathogenesis., Clinical Trial Registration: ClinicalTrials.gov identifier NCT04206553., (© 2024. Crown.)

Introduction: Linear IgA dermatosis (LAD) is a rare subepidermal autoimmune bullous disease (AIBD) defined by predominant or exclusive immune deposits of immunoglobulin A at the basement membrane zone of skin or mucous membranes. This disorder is a rare, clinically and immunologically heterogeneous disease occurring both in children and in adults. The aim of this project is to present the main clinical features of LAD, to propose a diagnostic algorithm and provide management guidelines based primarily on experts' opinion because of the lack of large methodologically sound clinical studies., Methods: These guidelines were initiated by the European Academy of Dermatology and Venereology (EADV) Task Force Autoimmune Bullous Diseases (AIBD). To achieve a broad consensus for these S2k consensus-based guidelines, a total of 29 experts from different countries, both European and non-European, including dermatologists, paediatric dermatologists and paediatricians were invited. All members of the guidelines committee agreed to develop consensus-based (S2k) guidelines. Prior to a first virtual consensus meeting, each of the invited authors elaborated a section of the present guidelines focusing on a selected topic, based on the relevant literature. All drafts were circulated among members of the writing group, and recommendations were discussed and voted during two hybrid consensus meetings., Results: The guidelines summarizes evidence-based and expert opinion-based recommendations (S2 level) on the diagnosis and treatment of LAD., Conclusion: These guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD., (© 2024 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Background: Patients with autoimmune blistering diseases (AIBDs) are often exposed to chronic glucocorticoid (GC) treatment with many side effects. Glucocorticoid-induced myopathy (GIM) is a well-established side effect, which particularly affects the proximal muscles. The Glucocorticoid Toxicity Index (GTI) is a validated global assessment tool which quantifies GC toxicity over time., Objectives: This study marks the first study which analyses GIM in patients with AIBDs. The objectives of this study were to utilize the GTI to investigate the nature and prevalence of GIM in AIBD patients and explore potential risk factors., Methods: This international cohort study was conducted in blistering disease clinics across Australia, China, Greece, Iran, Japan, the Philippines, Turkey and the United States of America between February 2019 and July 2023. The GTI tool was completed by a medical practitioner at each patient visit. Data related to glucocorticoid toxicity were entered into the Steritas GTI 2.0 to generate an aggregate improvement and cumulative worsening score at each visit., Results: The study included 139 patients. There were 132 episodes of myopathy, and 47.5% of patients developed muscle weakness at some point during the study period. Cumulative GC dose correlated positively with myopathy risk, while average dose and treatment duration were not significant. Older age, male gender and obesity more than doubled the likelihood of developing GIM., Conclusions: GIM is a common side effect experienced by AIBD patients on GC treatment. Muscle weakness is less likely to occur if cumulative GC dose is less than 0.75 mg/kg/day. Studies of exercise programs to mitigate myopathy and newer alternative treatments to reduce cumulative GC dose should be considered., (© 2024 The Author(s). Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Physicians and surgeons have a threefold increased risk of glioblastoma compared with population controls. We discuss the potential role of dermatology neurotoxin and carcinogenic occupational exposure, particularly to formalin/formaldehyde; how to reduce those exposures; and the ethical imperative for dermatologists to protect themselves, their staff, and their patients., Competing Interests: Declaration of competing interest There are no conflicts relevant to this contribution to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Hereditary hemochromatosis (HH) is a genetic disorder leading to excessive iron absorption, impacting multiple organs, notably the skin, nails and mucosae. The objective of this study is to elucidate the dermatologic manifestations, associated symptoms, pathophysiology and management recommendations of HH. We searched five primary databases (PubMed, Embase, Cochrane Library, Scopus and Web of Science) up to April 2023. Non-English articles were included to minimize language bias. The studies were evaluated using Oxford Centre for Evidence-based Medicine standards, with adherence to PRISMA guidelines. Inaccessible articles were directly sourced from authors. Out of the initial 1582 publications from 1904 to 2023, 22 studies (19 in English, 2 in French and 1 in German) were selected. Most reports were from the USA, UK and France and were predominantly case reports, covering 148 patients with skin symptoms related to hereditary hemochromatosis. We collected data on the cutaneous findings and, when available, their histopathological features. The current study highlights the scope, variety and traits of dermatologic symptoms in hereditary hemochromatosis, pinpointing research gaps and areas for future exploration. Our review accentuates the diverse dermatological manifestations of hereditary hemochromatosis, notably hyperpigmentation, hypertrichosis and resistant pruritus, often linked to excessive iron deposition and subsequent impairment of skin cell function. We also found controversial evidence indicating that skin cancers seem to be associated with hereditary hemochromatosis. Porphyria cutanea tarda and hereditary hemochromatosis were frequently reported together. Given hereditary hemochromatosis's genetic nature, early identification in one individual can substantially guide familial care and preemptive interventions. Clinicians should prioritize hereditary hemochromatosis as a differential when patients present with specific dermatological symptoms, especially in sun-exposed regions. A rigorous assessment ensures accurate diagnosis, facilitating optimal management for both the patient and their family., (© 2024 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Background: Inherited epidermolysis bullosa (EB) is a clinically and genetically heterogeneous group of skin fragility disorders characterized by blister formation following minor trauma. Four major types are distinguished based on the level of cleavage within the skin. Most EB forms present severely disabling cutaneous and systemic signs and symptoms. Management relies on daily time-consuming and distressing topical medications, and symptomatic treatment of systemic findings. Disease manifestations, symptoms, and daily care strongly affect patient and caregiver quality of life (QoL). To date, there are two validated EB-specific questionnaires, the "Quality of Life in Epidermolysis Bullosa" (QOLEB) and the "Epidermolysis Bullosa Burden of Disease" (EB-BoD) for the evaluation of patient and family disease burden, respectively. The aim of our study was to develop an Italian translation of the two questionnaires and to pilot-test them., Methods: The guidelines for translation and cross-cultural adaptation of health-related QoL measures were followed. Initially, two separate translations were generated for each questionnaire, and subsequently reconciled by an expert committee. This was followed by a back-translation process. The original texts and all translations underwent revision by the expert committee, resulting in definitive versions. The final versions were then tested in a pilot study involving cognitive debriefing in a group of 17 families, representative of all EB major types., Results: The translation and reconciliation process led to minor changes to obtain semantic/idiomatic/cultural equivalence of the Italian versions with the original ones and to reconcile the questions with the answer options. The cognitive debriefing process showed a good understanding and did not require text modifications., Conclusions: The Italian versions of the QOLEB and EB-BoD provide valuable tools in everyday clinical practice of reference centers, and they allow the participation in multicenter international real-life observational studies as well as in controlled clinical trials. They enable the identification of disease-specific psychological and socioeconomic challenges for EB patients and their families, guiding targeted interventions to ensure appropriate and timely care., (© 2024. The Author(s).)

Epidermolysis bullosa (EB) is a rare disease characterised by skin fragility and a wide variety of symptoms. The Quality of Life in Epidermolysis Bullosa (QOLEB) score is an English 17-item EB-specific validated measurement tool with two dimensions: functioning and emotions. The aim of this cross-sectional study was to develop and validate a culturally adapted German QOLEB. The following steps were carried out: translation, expert evaluation, back translation, linguistic and cultural adaptation, sample-based psychometric testing and evaluation. Data analysis was performed with n = 46 patients across all EB types. The reliability and internal consistency of the translated German QOLEB were excellent (α = 0.901). Regarding convergent validity, the QOLEB correlated highly with the iscorEB ( r = 0.879; p < 0.001). Structural similarity with the English original version was confirmed through exploratory factor analysis. In conclusion, the German QOLEB demonstrates internal reliability and construct validity and is suitable to assess the quality of life in German-speaking EB patients., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Competing Interests: Conflicts of interest D.F.M. is a principal investigator for Sun Pharma clinical trials. The other authors declare no conflicts of interest.

Autoimmune blistering diseases (AIBDs) comprise a group of rare conditions marked by autoantibodies that specifically target intercellular adhesion molecules. Despite the progress made in comprehending the disease and the increasing number of treatment options available, there is still no definitive cure for AIBDs such as pemphigus, and it continues to have a devastating impact on those affected. The challenges in achieving new approved therapies for AIBDs are complex and multifaceted. One significant obstacle was the prior lack of validated and standardized outcome measures, which are crucial for ensuring precise comparisons between new and traditional therapies. This gap in knowledge has prompted the development of minimal clinically important differences (MCIDs), which enable efficient and reliable comparison of therapeutic outcomes between trials. MCID is defined as the minimum difference in an outcome measure that indicates a clinically significant improvement/deterioration in disease severity. Additionally, MCIDs provide a patient-centered approach to evaluating treatment efficacy, by considering whether patients experience a subjective improvement in their symptoms. Therefore, this literature review will examine the derivation and significance of MCIDs for various scoring systems in AIBDs., Competing Interests: Author DM was a coinvestigator/cocreator of the PDAI, BPDAI and the EBDASI referenced in this article. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The handling editor TH is currently organizing a Research Topic with the author DM., (Copyright © 2023 Tseng, Stone and Murrell.)

Background: Scalp psoriasis affects most patients with psoriasis, but it can be difficult to treat., Objectives: To evaluate the efficacy and safety of once-daily roflumilast foam 0.3% on scalp and body psoriasis., Methods: In a phase IIb randomized controlled trial, adults and adolescents aged ≥ 12 years with scalp and body psoriasis were randomized (2 : 1) to roflumilast foam 0.3% or vehicle for 8 weeks. The primary efficacy endpoint was scalp Investigator Global Assessment (S-IGA) success (score of 'clear' or 'almost clear' plus ≥ 2-grade improvement from baseline) at week 8. Safety and tolerability were also evaluated., Results: Significantly more roflumilast-treated patients (59.1%) than vehicle-treated patients (11.4%) achieved S-IGA success at week 8 (P < 0.001); differences favoured roflumilast as early as the first postbaseline visit at week 2 (P < 0.001). Significant improvements were also seen for secondary endpoints, including body IGA success, Scalp Itch Numeric Rating Scale and the Psoriasis Scalp Severity Index. The safety of roflumilast was generally similar to vehicle. Patients treated with roflumilast experienced low rates of treatment-emergent adverse events (AEs), with few discontinuations due to an AE. Few patients with skin of colour (11%) and few adolescents (0.7%) were included., Conclusions: The results support the further development of roflumilast foam for treating scalp and body psoriasis., Competing Interests: Conflicts of interest: L.H.K. has received investigator fees, speaker fees, clinical research grants and/or honoraria from Abbott Laboratories, Ablynx, Acambis, Allergan, Almirall, Amgen, AnaptysBio, Aqua, Arcutis, Astellas Pharma US, Asubio Pharmaceuticals, Bayer Consumer Healthcare Pharmaceuticals, Beiersdorf, Biogen, BioLife, Biopelle, Boehringer Ingelheim, Botanix Pharmaceuticals, Breckenridge Pharma, Bristol Myers Squibb, Cassiopea, Celgene, Cellceutix, Centocor Ortho Biotech, ChemoCentryx, CollaGenex Pharmaceuticals, ColBar LifeScience, Connetics Corporation, Coria Laboratories, Dermavant Sciences, Dermik Laboratories (a business of Sanofi-Aventis US), Dow Pharmaceutical Sciences, DUSA Pharmaceuticals, Embil Pharmaceutical, EOS, Ferndale Laboratories, Galderma Laboratories, Genentech, GlaxoSmithKline, Healthpoint, Incyte Corporation, Intendis, Isdin, Johnson & Johnson Consumer Products Company, Laboratory Skin Care, LEO Pharma, 3M Pharmaceuticals, MC2 Therapeutics, Medical International Technologies, Medicis Pharmaceutical, Merck & Co., Merck-Serono, Merz Pharmaceuticals, NanoBio Corporation, Novartis Pharmaceuticals, Nucryst, Obagi Medical Products, Ortho Dermatologics, Onset Dermatologics, Pfizer, PharmaDerm, Promius Pharma, PuraCap Pharmaceutical, QLT, Sandoz (a Novartis company), SkinMedica, Stiefel Laboratories (a GSK company), Sun Pharmaceutical Industries, Taro Pharmaceutical Industries, TolerRx, Triax Pharmaceuticals, Valeant Pharmaceuticals International, Warner Chilcott, XenoPort and Zalicus. J.A.-L. is an investigator for Arcutis Biotherapeutics; a speaker for Celgene (Amgen), Dermira (Eli Lilly), Eli Lilly, Ortho Dermatologics and UCB Pharma; and serves on advisory boards for LEO Pharma. N.B. is an advisor, consultant and investigator for Arcutis Biotherapeutics, and received grants/research funding and honoraria. M.B. is an investigator for Arcutis Biotherapeutics, and received grants/research funding and honoraria. A.R.D. has received investigator fees, speaker fees and/or honoraria from AbbVie, Amgen, Arcutis Biotherapeutics, Bausch Health/Valeant Pharmaceuticals, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, Dermavant Sciences, Dermira, Eli Lilly, Galapagos, Galderma Laboratories, GSK, Incyte Corporation, Janssen Pharmaceuticals, LEO Pharma, Novartis, Pfizer, Regeneron Pharmaceuticals, Reistone Biopharma, Roche, Sanofi-Aventis/Genzyme, Sandoz, Sun Pharma, Takeda and UCB Pharma. Z.D.D. received a research grant from Arcutis Biotherapeutics to contribute to the data presented in the paper. J.D. is an investigator for and receives grants/research funding from AbbVie, AiViva BioPharma, Allergan, Almirall, AnaptysBio, Arcutis Biotherapeutics, Bausch Health, Bellus Medical, Biofrontera, Brickell Biotech, Bristol Myers Squibb, Caliway Biopharmaceuticals, Cara Therapeutics, Croma-Pharma, Dermata Therapeutics, DermBiont, Dr. Reddy’s Laboratories, Endo Pharmaceuticals, Evommune, Galderma USA, Hexima, Incyte Corporation, LEO Laboratories (LEO Pharma), Merck, NFlection Therapeutics, Palvella Therapeutics, RAPT Therapeutics and Therapeutics; is an advisory board member for and receives honoraria from RAPT Therapeutics; and is an independent contractor for and receives other financial benefit from Vial Health Technology. M.J.G. has received investigator fees, speaker fees and/or honoraria from AbbVie, Akros, Amgen, AnaptysBio, Arcutis Biotherapeutics, Aristea Therapeutics, Aslan Pharmaceuticals, Bausch, Bristol Myers Squibb, Boehringer Ingelheim, Celgene, Dermavant Sciences, Dermira, Eli Lilly, Galderma Laboratories, GSK, Incyte Corporation, Janssen Pharmaceuticals, Kyowa Kirin International, MedImmune, Meiji Seika Pharma, Merck, MoonLake Immunotherapeutics, Nimbus Therapeutics, Novartis, Pfizer, Regeneron Pharmaceuticals, Roche, Sun Pharma and UCB Pharma. S.E.K. declares no conflicts of interests. E.L. is an investigator for Arcutis Biotherapeutics, and has received grants/research funding and honoraria. M.L. is an investigator for AbbVie, Arcutis Biotherapeutics, AstraZeneca, Bausch Health, Boehringer Ingelheim, Boston Pharmaceuticals, Bristol Myers Squibb, Dermavant Sciences, Dermira, Eli Lilly, Incyte Corporation, Janssen Pharmaceuticals, Kiniksa Pharmaceuticals, LEO Pharma, Pfizer, RAPT Therapeutics, Reistone Biopharma, Regeneron Pharmaceuticals and UCB Biopharma. A.M. is an investigator for and has received research funds or honoraria from AbbVie, Arcutis Biotherapeutics, Bristol Myers Squibb, Dermavant Sciences, Eli Lilly, Galderma Laboratories, Janssen Pharmaceuticals and Nimbus Therapeutics. D.F.M. is an advisory board member/investigator for Almirall, Amicus Therapeutics, Amgen, Amryt Pharma, AnaptysBio, Arcutis Biotherapeutics, Arena Pharmaceuticals, ArgenX, AstraZeneca, Azora Therapeutics, Castle Creek Biosciences, Dermira, Eli Lilly, Evelo Biosciences, Factor Bioscience, Galderma Laboratories, Innovaderm Research, Janssen Pharmaceuticals, LEO Pharma, Novartis, Pfizer, Principia Biopharma, Regeneron Pharmaceuticals, Rheacell, Sanofi, Shire, Sienna Biopharmaceuticals, Sun Pharma, UCB Pharma and Xerion Healthcare. K.A.P. has received investigator fees, speaker fees, clinical research grants and/or honoraria from AbbVie, Akros, Amgen, Anacor Pharmaceuticals, Arcutis Biotherapeutics, Astellas Pharma US, Avillion, Bausch Health/Valeant Pharmaceuticals International, Baxalta, Boehringer Ingelheim, Bristol Myers Squibb, Can-Fite BioPharma, Celgene, Celltrion Healthcare, Coherus Biosciences, Dermavant Sciences, Dermira, Dice Pharmaceuticals, Dow Pharma, Eli Lilly, Evelo Biosciences, Forbion, Galapagos, Galderma Laboratories, Genentech, Gilead, GSK, Incyte Corporation, Janssen Pharmaceuticals, Kyowa Hakko Kirin, LEO Pharma, Medimmune, Meiji Seika Pharma, Merck (MSD), Merck-Serono, Mitsubishi Pharma, Moberg Pharma, Novartis, Pfizer, PRCL Research, Regeneron Pharmaceuticals, Reistone Biopharma, Roche, Sanofi-Aventis/Genzyme, Sandoz, Sun Pharma, Takeda, UCB Pharma, vTv Therapeutics and Xencor. D.M.P. is an investigator for and receives grants/research funding and/or honoraria from Almirall, Amgen, AOBiome, Asana BioSciences, Bickel Biotechnology, Celgene Corporation, Dermira, Eli Lilly and Company, LEO Pharma, Menlo Therapeutics, Novartis Pharmaceuticals, Novo Nordisk, Ortho Dermatologics, Pfizer and Regeneron Pharmaceuticals; is a consultant for and/or serves on an advisory board for and receives honoraria from Bickel Biotechnology, Biofrontera, Dermira, Novartis Pharmaceuticals, Pfizer, Regeneron Pharmaceuticals and Sanofi; and is on the Data Safety Monitoring Board for and receives honoraria from Bristol Myers Squibb. R.S. is an investigator for Arcutis Biotherapeutics and received grants/research funding and honoraria. M.Z. has received consulting fees, investigator fees, speaker fees, clinical research grants or sponsored research, and/or honoraria from AbbVie, All Free Clear/Sun, AnaptysBio, Arcutis Biotherapeutics, Asana BioSciences, Aseptic, Avillion, Bausch Health, Cara Therapeutics, Concert Pharmaceuticals, Dermavant Sciences, Edesa Biotech, Eli Lilly, EPI Health, Fitbit, Galderma Laboratories, Genentech/Novartis, Incyte Corporation, Janssen Pharmaceuticals, LEO Pharma, Level Ex, L’Oréal, LUUM, Oculus, Peloton, Pfizer, Regeneron Pharmaceuticals/Sanofi, Sol-Gel, Trevi Therapeutics, UCB Pharma and VYNE Therapeutics. P.B., R.C.H., D.K. and D.R.B. are employees of Arcutis Biotherapeutics. This work was supported by Arcutis Biotherapeutics., (© The Author(s) 2023. Published by Oxford University Press on behalf of British Association of Dermatologists.)

Competing Interests: Conflicts of interest None disclosed.

Background: Dermatology journals play an essential role in the distribution and promotion of scientific and medical information. Despite this, there are little data on governance structure with respect to its editors, owners, and journal boards that oversee the day-to-day operations for these entities., Objective: This study aimed to explore the current governance structure of dermatology journals and best practice recommendations., Methods: The editors-in-chief of the major dermatology journals participated in an online survey of 29 questions to examine general statistics of each journal, open access model, governance structure, and process for editor selection or dismissal., Results: Of the 52 journal responses, 29 (55.8%) are society-owned journals with 19 (65.5%) primarily governed by a society board, while 18 (34.6%) have an advisory committee or alternative body. Most editor(s)-in-chief (56.9%) serve between 3- and 5-year terms, while 84.6% have the option of at least one renewal. Even though the selection, evaluation, and dismissal processes differed between the journals, generalized best practice recommendations were developed to help improve their overall organization and management., Conclusions: The oversight structure of dermatology journals varies, and some do not follow current best practice recommendations. Transparency regarding leadership, governance, and due process is needed to maintain editorial independence and integrity., Competing Interests: None disclosed., (© 2023 by the American Academy of Dermatology, Inc. Published by Elsevier Inc.)

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disease. Oral steroids are the standard treatment. We have updated this review, which was first published in 2002, because several new treatments have since been tried., Objectives: To assess the effects of treatments for bullous pemphigoid., Search Methods: We updated searches of the following databases to November 2021: Cochrane Skin Specialised Register, CENTRAL, MEDLINE, and Embase. We searched five trial databases to January 2022, and checked the reference lists of included studies for further references to relevant randomised controlled trials (RCTs)., Selection Criteria: RCTs of treatments for immunofluorescence-confirmed bullous pemphigoid., Data Collection and Analysis: At least two review authors, working independently, evaluated the studies against the review's inclusion criteria and extracted data from included studies. Using GRADE methodology, we assessed the certainty of the evidence for each outcome in each comparison. Our primary outcomes were healing of skin lesions and mortality., Main Results: We identified 14 RCTs (1442 participants). The main treatment modalities assessed were oral steroids, topical steroids, and the oral anti-inflammatory antibiotic doxycycline. Most studies reported mortality but adverse events and quality of life were not well reported. We decided to look at the primary outcomes 'disease control' and 'mortality'. Almost all studies investigated different comparisons; two studies were placebo-controlled. The results are therefore based on a single study for each comparison except azathioprine. Most studies involved only small numbers of participants. We assessed the risk of bias for all key outcomes as having 'some concerns' or high risk, due to missing data, inappropriate analysis, or insufficient information. Clobetasol propionate cream versus oral prednisone Compared to oral prednisone, clobetasol propionate cream applied over the whole body probably increases skin healing at day 21 (risk ratio (RR 1.08, 95% confidence interval (CI) 1.03 to 1.13; 1 study, 341 participants; moderate-certainty evidence). Skin healing at 21 days was seen in 99.8% of participants assigned to clobetasol and 92.4% of participants assigned to prednisone. Clobetasol propionate cream applied over the whole body compared to oral prednisone may reduce mortality at one year (RR 0.73, 95% CI 0.53 to 1.01; 1 study, 341 participants; low-certainty evidence). Death occurred in 26.5% (45/170) of participants assigned to clobetasol and 36.3% (62/171) of participants assigned to oral prednisone. This study did not measure quality of life. Clobetasol propionate cream may reduce risk of severe complications by day 21 compared with oral prednisone (RR 0.65, 95% CI 0.50 to 0.86; 1 study, 341 participants; low-certainty evidence). Mild clobetasol propionate cream regimen (10 to 30 g/day) versus standard clobetasol propionate cream regimen (40 g/day) A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen probably does not change skin healing at day 21 (RR 1.00, 95% CI 0.97 to 1.03; 1 study, 312 participants; moderate-certainty evidence). Both groups showed complete healing of lesions at day 21 in 98% participants. A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen may not change mortality at one year (RR 1.00, 95% CI 0.75 to 1.32; 1 study, 312 participants; low-certainty evidence), which occurred in 118/312 (37.9%) participants. This study did not measure quality of life. A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen may not change adverse events at one year (RR 0.94, 95% CI 0.78 to 1.14; 1 study, 309 participants; low-certainty evidence). Doxycycline versus prednisolone Compared to prednisolone (0.5 mg/kg/day), doxycycline (200 mg/day) induces less skin healing at six weeks (RR 0.81, 95% CI 0.72 to 0.92; 1 study, 213 participants; high-certainty evidence). Complete skin healing was reported in 73.8% of participants assigned to doxycycline and 91.1% assigned to prednisolone. Doxycycline compared to prednisolone probably decreases mortality at one year (RR 0.25, 95% CI 0.07 to 0.89; number needed to treat for an additional beneficial outcome (NNTB) = 14; 1 study, 234 participants; moderate-certainty evidence). Mortality occurred in 2.4% (3/132) of participants with doxycycline and 9.7% (11/121) with prednisolone. Compared to prednisolone, doxycycline improved quality of life at one year (mean difference 1.8 points lower, which is more favourable on the Dermatology Life Quality Index, 95% CI 1.02 to 2.58 lower; 1 study, 234 participants; high-certainty evidence). Doxycycline compared to prednisolone probably reduces severe or life-threatening treatment-related adverse events at one year (RR 0.59, 95% CI 0.35 to 0.99; 1 study, 234 participants; moderate-certainty evidence). Prednisone plus azathioprine versus prednisone It is unclear whether azathioprine plus prednisone compared to prednisone alone affects skin healing or mortality because there was only very low-certainty evidence from two trials (98 participants). These studies did not measure quality of life. Adverse events were reported in a total of 20/48 (42%) participants assigned to azathioprine plus prednisone and 15/44 (34%) participants assigned to prednisone. Nicotinamide plus tetracycline versus prednisone It is unclear whether nicotinamide plus tetracycline compared to prednisone affects skin healing or mortality because there was only very low-certainty evidence from one trial (18 participants). This study did not measure quality of life. Fewer adverse events were reported in the nicotinamide group. Methylprednisolone plus azathioprine versus methylprednisolone plus dapsone It is unclear whether azathioprine plus methylprednisolone compared to dapsone plus methylprednisolone affects skin healing or mortality because there was only very low-certainty evidence from one trial (54 participants). This study did not measure quality of life. A total of 18 adverse events were reported in the azathioprine group and 13 in the dapsone group., Authors' Conclusions: Clobetasol propionate cream applied over the whole body is probably similarly effective as, and may cause less mortality than, oral prednisone for treating bullous pemphigoid. Lower-dose clobetasol propionate cream applied over the whole body is probably similarly effective as standard-dose clobetasol propionate cream and has similar mortality. Doxycycline is less effective but causes less mortality than prednisolone for treating bullous pemphigoid. Other treatments need further investigation., (Copyright © 2023 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.)

Disease severity assessment tools play a large part in evaluating skin conditions in dermatology. Currently, there is no existing validated assessment tool for keratosis pilaris (KP), a benign yet highly prevalent follicular disorder. A range of proposed scoring tools have been used in different clinical trials for the assessment of potential treatments for KP. A literature review of the current scoring systems used for KP shows that there is a lack of consistency with most studies using varying versions of unvalidated investigator global assessment (IGA) scores and quartile grading systems. A review of these studies shows that current methods of evaluating KP in clinical trials are subjective, unreliable, and inconsistent. A standardised and validated scoring system would be significant as it could be used in clinical trials to advance the current knowledge of KP., Competing Interests: The authors have no conflicts of interest to declare., (© 2023 S. Karger AG, Basel.)

Background: Emollients and keratolytics are frequently used to manage symptoms of congenital ichthyosis (CI). Systemic retinoid treatment is complicated by teratogenicity and dose-limiting adverse effects., Objectives: This analysis from the randomized Phase IIb CONTROL study investigated the characteristics of participants who responded to treatment with TMB-001, a novel topical isotretinoin ointment formulation., Methods: Participants ≥ 9 years of age with genetically confirmed CI and ≥ 2 (out of 4) Visual Index for Ichthyosis Severity (VIIS) assessment areas with ≥ 3 scaling score were randomized 1 : 1 : 1 to TMB-001 0.05%, TMB-001 0.1% or vehicle, twice daily for 12 weeks. Efficacy endpoints included the proportion of participants with ≥ 50% reduction in VIIS-scaling (VIIS-50) compared with baseline and ≥ 2-grade reduction in Investigator's Global Assessment (IGA)-scaling score compared with baseline. Changes in body surface area (BSA) involvement, Dermatology Life Quality Index (DLQI) scores and Itch-Numeric Rating Scale (I-NRS) scores were assessed., Results: Among the 33 participants (11 randomized to TMB-001 0.05%, 10 to TMB-001 0.1% and 12 to vehicle), median age was 29 years (range 9-80), and most were male (64%) and White (79%). Baseline demographics were generally similar among participants who did or did not achieve TMB-001 treatment success. Participants who had lower mean BSA involvement and higher DLQI and I-NRS scores at baseline were more likely to achieve VIIS-50. Similarly, higher baseline DLQI and I-NRS scores were associated with IGA response; BSA involvement was similar for IGA responders vs. nonresponders., Conclusions: Higher DLQI and I-NRS scores at baseline were associated with participants achieving treatment success by VIIS-50 and IGA response. Lower BSA involvement was associated with VIIS-50 success., (© The Author(s) 2023. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Etrasimod is an oral, selective, sphingosine 1-phosphate (S1P) receptor 1,4,5 modulator in development for immune-mediated inflammatory disorders. Efficacy and safety of orally administered S1P receptor modulation in atopic dermatitis (AD) have not yet been examined., Objective: To assess the efficacy and safety of etrasimod monotherapy in adults with moderate-to-severe AD., Methods: In this phase 2, randomized, double-blind, placebo-controlled trial, participants (≥18 years) with moderate-to-severe AD defined as baseline validated Investigator's Global Assessment (vIGA-AD) score ≥ 3, Eczema Area and Severity Index (EASI) score ≥ 16, and body surface area involvement ≥10% were randomized 1:1:1 to once-daily oral etrasimod 1 mg, 2 mg or placebo for 12 weeks. The primary outcome was percent change in EASI score from baseline at week 12, assessed in the Full Analysis Set (all randomized participants). Key secondary outcomes were achievement of a vIGA-AD score of 0 or 1 with a ≥2-point improvement from baseline and EASI-75 response at Week 12. Safety was assessed during the double-blind period., Results: One hundred and forty participants were randomized to etrasimod 2 mg (n = 47), 1 mg (n = 47) or placebo (n = 46). At Week 12, percent change in EASI score was -57.2% in the etrasimod 2-mg group versus -48.4% in the placebo group (p = 0.18). A significantly greater proportion of participants receiving etrasimod 2 mg achieved vIGA-AD scores of 0 or 1 with a ≥2-point improvement at Week 12 versus placebo (29.8% vs. 13.0%; p = 0.045); however, EASI-75 response was not statistically significant versus placebo. Treatment-emergent adverse events (AEs) occurred in 59.6%, 40.4% and 47.8% of participants receiving etrasimod 2 mg, 1 mg and placebo, respectively. There were no serious AEs or deaths., Conclusions: The primary outcome was not met, although efficacy was observed for etrasimod 2 mg on several clinician- and patient-assessed measures, and both 1- and 2-mg doses were well tolerated, warranting further clinical investigation in AD., (© 2023 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Background: In two severe congenital ichthyosis subtypes, autosomal recessive lamellar ichthyosis (ARCI-LI) and X-linked recessive ichthyosis (XLRI), cutaneous manifestations include widespread scaling. Approved topical treatment options are limited to emollients and keratolytics., Aim: This analysis from the randomized phase IIb CONTROL study assessed whether the efficacy and safety of TMB-001, a novel topical isotretinoin ointment formulation, differed between ARCI-LI and XLRI subtypes., Methods: Participants ≥ 9 years with genetically confirmed XLRI or ARCI-LI and ≥ 2 (of 4) Visual Index for Ichthyosis Severity (VIIS) assessment areas with ≥ 3 scaling score were randomized 1 : 1 : 1 to TMB-001 0.05%/TMB-001 0.1%/vehicle, twice daily for 12 weeks. The proportion of participants with ≥ 50% reduction vs. baseline in VIIS scaling (VIIS 50; primary endpoint) and ≥ 2-grade reduction in Investigator's Global Assessment (IGA)-scaling score vs. baseline (key secondary endpoint) were evaluated. Adverse events (AEs) were monitored., Results: Among enrolled participants (TMB-001 0.05%, n = 11; 0.1%, n = 10; and vehicle, n = 12), 52% had ARCI-LI and 48% XLRI subtypes. Mean age was 33.6 and 35.4 years for participants with ARCI-LI and XLRI, respectively. Overall, 33%, 50% and 17% of participants with ARCI-LI and 100%, 33% and 75% of participants with XLRI achieved VIIS 50 in the TMB-001 0.05%, TMB-001 0.1% and vehicle groups, respectively (nominal P = 0.24 for 0.05% vs. vehicle, intent-to-treat population). Improvement of ≥ 2-grade IGA score was observed in 33%, 50% and 0% of participants with ARCI-LI and 83%, 33% and 25% of participants with XLRI in the TMB-001 0.05%, TMB-001 0.1% and vehicle groups, respectively (nominal P = 0.03 for 0.05% vs. vehicle, intention-to-treat population). Most AEs were application-site reactions., Conclusion: Regardless of congenital ichthyosis subtype, TMB-001 demonstrated greater proportions of participants achieving VIIS 50 and ≥ 2-grade IGA improvement vs. vehicle., Competing Interests: Conflicts of interest D.F.M. has served an investigator and adviser for AbbVie; Amgen; ArgenX; Dermira; Eli Lilly and Company; Galderma; Janssen; LEO Pharma; Novartis; Pfizer; Principia Biopharma; Roche; Sanofi; and Sun Pharmaceuticals, Inc.; and has served on an advisory board for Eli Lilly and Company, Immune Pharmaceuticals and Principia Biopharma. J.M.C.T. has served as an investigator for Castle Creek Pharmaceuticals, LEO Pharma, Novartis, Pfizer Inc., Palvella Therapeutics, Regeneron, and Timber Pharmaceuticals; and a consultant for Abeona Therapeutics; AFT Pharma; Amryst; BridgeBio; Castle Creek Pharmaceuticals; KrystalBio; LEO Pharma; Menlo Therapeutics; Nobelpharma Co., Ltd; Novartis; Pfizer Inc; Palvella Therapeutics; Regeneron; and Timber Pharmaceuticals. S.G. and K.E. have served as scientific advisers and/or clinical study investigators for AbbVie; Aclaris; Almirall; Amgen; AOBiome; Anaptys Bio; Arcutis; Asana; Atacama; Athenex; Boehringer Ingelheim; Bristol Myers Squibb; Cara; Cassiopeia; Celgene; Concert Pharmaceuticals; Crown Therapeutics; Cutanea; Dermavant; Dermira; Edesa; Eli Lilly and Company; Foamix; Forte; Gage; Galderma; Glenmark; Incyte; Janssen; LEO Pharma; Novan; Novartis; Novum; Pfizer Inc.; Rapt; Regeneron; Sanofi Genzyme; Sun Pharmaceutical Inc; Timber Pharmaceuticals; Vanda; Verrica; Vidac; and UCB Pharma. K.M. has served as an adviser for Janssen. S.K. served as a consultant for Arcutis Biotherapeutics; and a clinical trial investigator for AbbVie, Amgen, Arcutis Biotherapeutics, Brickell Biotech, Bristol Myers Squibb, Candesant Biomedical, Concert Pharmaceuticals, Dermira, Edesa Biotech, Eli Lilly and Company, Forte Biosciences, Galderma, Incyte Corporation, NFlection Therapeutics, Novan, Novartis, Palvella Therapeutics and Timber Pharmaceuticals. L.C.-S. received honoraria from and served on an advisory panel for Pfizer Inc; and served as clinical study investigator for Timber Pharmaceuticals. A.M.M. and J.R. are employees of Timber Pharmaceuticals. C.G.B. has served as an investigator for Almirall; a consultant for AbbVie, Almirall, Arcutis, LEO Pharma, Sanofi-Regeneron, Skinosive, and UCB; and a speaker for and received honoraria from Allergan, Almirall, LEO Pharma and UCB., (Published by Oxford University Press on behalf of British Association of Dermatologists 2023.)

Competing Interests: None disclosed.

Background: In epidermolysis bullosa simplex (EBS), epithelial structural fragility results in blisters and erosions. Diacerein 1% ointment has been shown to reduce this blistering., Objective: To evaluate the efficacy and safety of diacerein 1% ointment in the treatment of EBS., Methods: A double-blind study of 54 patients with EBS were randomized to diacerein 1% or vehicle ointment once daily. The primary endpoint ( &ge;60% reduction in body surface area of EBS) and the key secondary endpoint ( &ge;2-point reduction in the Investigator&rsquo;s Global Assessment) were evaluated at 8 weeks., Results: There was no difference in the proportion of patients achieving either key efficacy endpoint between the diacerein 1% and vehicle groups (P&gt;0.05). No difference in treatment emergent adverse events were noted between the groups. In post hoc analysis stratified by EBS subtypes, an IGA score of 0 or 1 was reported in 6 of 13 patients with severe EBS in the diacerein group (46.2%), compared with 2 of 13 patients with severe EBS in the vehicle group (15.4%); (relative risk= 3.08, 95% CI = 0.71, 13.4)., Conclusions: Although there was no significant difference in outcomes between the groups, further study may elucidate the effects of diacerein on EBS lesions, especially in patients with severe EBS. Teng J, Paller AS, Bruckner AL, et al. Diacerein 1% ointment for the treatment of epidermolysis bullosa simplex: a randomized, controlled trial. J Drugs Dermatol. 2023;22(6):599-604. doi:10.36849/JDD.7108.