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Introduction: Alopecia areata (AA) is an autoimmune disease associated with high rates of emotional and psychosocial distress. The analysis reported here describes the evolution of measures assessing health-related quality of life (HRQoL) and symptoms of anxiety and depression up to week 104 in patients who achieved sustained scalp hair regrowth during treatment with baricitinib in the BRAVE-AA phase III trials., Methods: This post-hoc analysis included data from the double-blind, parallel-group, randomized, placebo-controlled phase III trials BRAVE-AA1 (ClinicalTrials.gov number: NCT03570749) and BRAVE-AA2 (ClinicalTrials.gov number: NCT03899259). Adults with severe AA (defined as a Severity of Alopecia Tool [SALT] score ≥ 50) randomized to baricitinib 4 mg or baricitinib 2 mg at baseline who achieved SALT score ≤ 20 by week 36 and maintained SALT score ≤ 20 through week 104 on the same dose of baricitinib were included in this analysis of integrated data. Scalp hair regrowth (SALT score) and improvements in Skindex-16 AA Scale and Hospital Anxiety and Depression Scale (HADS) domain scores were analyzed over the 104-week period using descriptive statistics., Results: In total, 131 patients (88 on baricitinib 4 mg and 43 on baricitinib 2 mg) were included in this analysis. Across the two groups, the mean age (standard deviation) was 37.2 years (12.7), and 84 (64.1%) patients were female. The interquartile range) for time to achieve a SALT score ≤ 20 for patients treated with baricitinib 4 mg and baricitinib 2 mg was 13.1 and 19.6 weeks, respectively. By week 104, 91% (baricitinib 2 mg) and 96% (baricitinib 4 mg) of patients had achieved a SALT score ≤ 10 on baricitinib treatment. In both groups, progressive improvements in the Skindex-16 AA and HADS domain scores were observed up to week 104., Conclusion: This analysis of adults with severe AA treated with baricitinib revealed that achievement of sustained clinically meaningful scalp hair regrowth (SALT score ≤ 20) was associated with improvements in both measures of HRQoL and symptoms of anxiety and depression up to week 104., (© 2024. The Author(s).)

Background: Ichthyoses are a heterogeneous group of skin disorders characterized by scaling and erythema. Recognizing the variability of scale and erythema by region and ichthyosis subtype, we developed the Ichthyosis Scoring System (ISS) to quantify severity. We previously found ISS to have high inter- and intrarater reliability in evaluating photographic images. To confirm ISS clinical utility, we examined its performance at the 2022 Foundation for Ichthyosis and Related Skin Types conference., Methods: Sixty-five participants were evaluated by 3 of 9 medical professionals trained to score ichthyosis scale and erythema using ISS. Intrarater and interrater intraclass correlation coefficients (ICC) were analyzed using one-way and two-way random effects models, respectively., Results: Intrarater reliability was excellent (ICC = 0.931, 95% CI, 0.921-0.940) for scale and good (ICC = 0.876, 95% CI, 0.853-0.899) for erythema scoring. Compared to photo validation with excellent intrarater reliability ratings for both scale (ICC = 0.956, 95% CI, 0.925-0.974) and erythema (ICC = 0.913, 95% CI, 0.855-0.949), ISS demonstrated equivalent reliability for live use. Overall interrater reliability for 10 body sites showed excellent (ICC >0.9) and good (ICC >0.75) agreement and consistency for both scale and erythema. Palms were an exception, demonstrating moderate (ICC >0.5) interrater agreement and consistency for erythema evaluation., Conclusions: ISS is a reliable measure of global and regional ichthyosis severity during in-person evaluations. Ease-of-use, accessibility, and content validity in both live and photographic evaluation endorse ISS as a standard for ichthyosis severity analysis., (© 2024 Wiley Periodicals LLC.)

Importance: Current measures of alopecia areata (AA) severity, such as the Severity of Alopecia Tool score, do not adequately capture overall disease impact., Objective: To explore factors associated with AA severity beyond scalp hair loss, and to support the development of the Alopecia Areata Severity and Morbidity Index (ASAMI)., Evidence Review: A total of 74 hair and scalp disorder specialists from multiple continents were invited to participate in an eDelphi project consisting of 3 survey rounds. The first 2 sessions took place via a text-based web application following the Delphi study design. The final round took place virtually among participants via video conferencing software on April 30, 2022., Findings: Of all invited experts, 64 completed the first survey round (global representation: Africa [4.7%], Asia [9.4%], Australia [14.1%], Europe [43.8%], North America [23.4%], and South America [4.7%]; health care setting: public [20.3%], private [28.1%], and both [51.6%]). A total of 58 specialists completed the second round, and 42 participated in the final video conference meeting. Overall, consensus was achieved in 96 of 107 questions. Several factors, independent of the Severity of Alopecia Tool score, were identified as potentially worsening AA severity outcomes. These factors included a disease duration of 12 months or more, 3 or more relapses, inadequate response to topical or systemic treatments, rapid disease progression, difficulty in cosmetically concealing hair loss, facial hair involvement (eyebrows, eyelashes, and/or beard), nail involvement, impaired quality of life, and a history of anxiety, depression, or suicidal ideation due to or exacerbated by AA. Consensus was reached that the Alopecia Areata Investigator Global Assessment scale adequately classified the severity of scalp hair loss., Conclusions and Relevance: This eDelphi survey study, with consensus among global experts, identified various determinants of AA severity, encompassing not only scalp hair loss but also other outcomes. These findings are expected to facilitate the development of a multicomponent severity tool that endeavors to competently measure disease impact. The findings are also anticipated to aid in identifying candidates for current and emerging systemic treatments. Future research must incorporate the perspectives of patients and the public to assign weight to the domains recognized in this project as associated with AA severity.

Introduction: This post hoc analysis assessed association between scalp hair regrowth and improvements in health-related quality of life (HRQoL) and psychological burden in patients with severe alopecia areata (AA)., Methods: Data were pooled from two phase-3 trials ( N  = 1200). Patients randomized to once-daily placebo, baricitinib 2-mg, or 4-mg were analyzed independently of treatment allocation, and categorized according to scalp hair regrowth (at Week 36): meaningful regrowth (Severity of Alopecia Tool (SALT) score ≤20); intermediate regrowth (≥30% SALT improvement [SALT 30 ] at any post-baseline visit to Week 36, but SALT score > 20 at Week 36); no/minimal regrowth (never achieved SALT 30 ). Skindex-16 for AA score change-from-baseline and proportion of patients with baseline Hospital Anxiety and Depression Scale (HADS) scores ≥8 that shifted to <8 (normal) were assessed., Results: Patients with meaningful regrowth achieved greater improvements in all Skindex-16 AA domains versus no/minimal regrowth. More patients with meaningful versus no/minimal regrowth shifted from HADS ≥8 to <8 (anxiety:46.8% versus 26.4%; depression:52.3% versus 24.0%). Improvements occurred with intermediate regrowth but to a lesser extent versus meaningful regrowth., Conclusions: Patients with severe AA and scalp hair regrowth at Week 36 experienced greater improvements in HRQoL and anxiety and depression versus patients with no/minimal regrowth. The highest benefit was observed in patients with meaningful regrowth (SALT score ≤20). ClinicalTrials.gov listing: NCT03570749 and NCT03899259.

Background: Baricitinib is approved for the treatment of adults with severe alopecia areata (AA). In the absence of robust data on the patterns of regrowth during treatment of severe AA, there is a gap in the knowledge regarding treatment expectations., Objectives: To examine whether different clinical response subgroups could be identified in baricitinib-treated patients with severe AA and factors that contribute to these subgroups., Methods: The BRAVE-AA1 and BRAVE-AA2 phase III trials enrolled patients with severe AA [Severity of Alopecia Tool (SALT) score ≥ 50 (≥ 50% scalp hair loss)]. Patients randomized to baricitinib 4 mg or 2 mg retained their treatment allocation for 52 weeks. Based on patterns identified through growth mixture modelling (GMM), patients were categorized into responder subgroups according to when they first achieved ≥ 30% improvement from baseline in SALT score (SALT30). For each responder subgroup, trajectories of response (i.e. achievement of a SALT score ≤ 20, SALT score ≤ 10 and ≥ 50% change from baseline in SALT score) and baseline disease characteristics are reported., Results: Respectively, 515 and 340 patients were randomized to once-daily baricitinib 4 mg and 2 mg at baseline; 69% and 51%, respectively, achieved SALT30 at least once by week 52. Based on GMM findings, we identified three responder subgroups: early (SALT30 by week 12), gradual (SALT30 after week 12-week 36) and late (SALT30 after week 36-week 52). The proportions of early, gradual and late responders and nonresponders were, respectively, 33%, 28%, 8% and 31% among patients treated with baricitinib 4 mg, and 20%, 23%, 9% and 49%, respectively, among those treated with baricitinib 2 mg. Early responders had a shorter trajectory to maximal clinical outcomes (e.g. > 78% achieved a SALT score ≤ 20 by week 36) vs. gradual or late responders. Early responders were more frequent among patients with baseline severe AA (SALT score 50 to < 95) vs. very severe AA (SALT score 95-100). Overall, responders (early to late) were more frequent in patients with short (< 4 years) episodes of hair loss., Conclusions: These analyses identified early, gradual and late responder subgroups for scalp hair regrowth in baricitinib-treated patients with severe AA, and that these subgroups are influenced by baseline characteristics. Findings from these analyses will help to inform treatment expectations for scalp hair regrowth., Competing Interests: Conflicts of interest B.K. has served on advisory boards and/or is a consultant and/or is a clinical trial investigator for AbbVie, AltruBio, Almirall, AnaptysBio, Arena Pharmaceuticals, Bioniz Therapeutics, Bristol Myers Squibb, Concert Pharmaceuticals, Equillium, Horizon Therapeutics, Eli Lilly and Company, Incyte, Janssen Pharmaceuticals, LEO Pharma, Otsuka/Visterra, Pfizer, Regeneron, Sanofi-Genzyme, TWi Biotechnology and Viela Bio; and has served on speaker bureaus for AbbVie, Eli Lilly and Company, Incyte, Pfizer, Regeneron and Sanofi Genzyme. J.S. has received travel reimbursement and speaking honoraria from Eli Lilly and Company. M.O. has received lecture fees from Eli Lilly and Company and advisory fees from Eli Lilly and Company, Pfizer, Janssen Pharmaceuticals (Japan), Taisho Pharmaceutical, Maruho, Bristol Myers Squibb Japan, AbbVie and ROHTO Pharmaceutical; and grants/research funds from Shiseido, Maruho, Advantest and Sun Pharma Japan. He is a specialist associate editor of the British Journal of Dermatology and was excluded from all editorial decision-making related to the acceptance of this article for publication. A.E. has received research funding from Pfizer, Eli Lilly and Company, Novartis, Bristol Myers Squibb, Boehringer Ingelheim, AbbVie, Janssen Pharmaceuticals, Boehringer Ingelheim, the Danish National Psoriasis Foundation, the Simon Spies Foundation and the Kgl Hofbundtmager Aage Bang Foundation; and honoraria as a consultant and/or speaker from AbbVie, Almirall, Amgen, Boehringer Ingelheim, LEO Pharma, Zuellig Pharma, Galápagos, Sun Pharmaceuticals, Samsung Bioepis, Pfizer, Eli Lilly and Company, Novartis, Union Therapeutics, Galderma, Dermavant, UCB, Mylan, Bristol Myers Squibb, McNeil Consumer Healthcare, Horizon Therapeutics, Boehringer Ingelheim and Janssen Pharmaceuticals. B.M.P. has received lecture fees from Eli Lilly and Company and Pfizer; consulting fees from Eli Lilly and Company, Pfizer, ISDIN, Almirall and Vichy; and has participated in advisory boards for Eli Lilly and Company and Pfizer. B.C. has received honoraria and/or fees from Eli Lilly and Company, Pfizer, Regeneron and Sanofi-Genzyme. R.S. reports serving as a consultant or paid speaker for or participating in clinical trials sponsored by LEO Pharma, Amgen, Novartis Pharmaceuticals, Merck & Co, Celgene, Coherus BioSciences, Janssen Global Services, Regeneron Pharmaceuticals, MedImmune, GlaxoSmithKline, Cutanea, Samson Clinical, Boehringer Ingelheim, Pfizer, Merck Sharpe & Dohme, Oncobiologics, F. Hoffmann-La Roche, Eli Lilly and Company, and Bayer; and is serving as the current President of the Australasian Hair and Wool Research Society. Y.-F.C., W.-S.W, Y.D., N.S. and Y.D. are employees of and shareholders in Eli Lilly and Company., (© The Author(s) 2023. Published by Oxford University Press on behalf of British Association of Dermatologists.)

Introduction: The physical impact of alopecia areata (AA) is visible, but the psychological and social consequences and emotional burden are often underrecognized., Methods: In this cross-sectional study, 547 participants recruited via the National Alopecia Areata Foundation completed a survey encompassing demographics; AA illness characteristics; and five patient-reported outcome measures on anxiety and depression, perceived stress, psychological illness impact, stigma, and quality of life (QoL). Differences in disease severity subgroups were assessed via analysis of variance (ANOVA) and t tests., Results: Mean age was 44.6 years, and 76.6% were female. Participants with more severe hair loss tended to report longer duration of experiencing AA symptoms (P < 0.001). Overall, participants reported negative psychological impact, emotional burden, and poor QoL due to AA. Participants with 21-49% or 50-94% scalp hair loss reported greater psychological impact and poorer QoL than those with 95-100% scalp hair loss (most parameters P < 0.05). Similar results were observed for eyebrow/eyelash involvement subgroups., Conclusions: These results suggest that participants with AA experience emotional burden, negative self-perception, and stigma, but the impact of AA is not dependent solely on the amount of hair loss. Lower impact among participants with 95-100% scalp hair loss may indicate that they have adapted to living with AA., (© 2023. The Author(s).)

Background: The current classification for alopecia areata (AA) does not provide a consistent assessment of disease severity., Objective: To develop an AA severity scale based on expert experience., Methods: A modified Delphi process was utilized. An advisory group of 22 AA clinical experts from the United States was formed to develop this AA scale. Representatives from the pharmaceutical industry provided feedback during its development., Results: Survey responses were used to draft severity criteria, aspiring to develop a simple scale that may be easily applied in clinical practice. A consensus vote was held to determine the final AA severity statement, with all AA experts agreeing to adopt the proposed scale., Limitations: The scale is a static assessment intended to be used in clinical practice and not clinical trials., Conclusion: The final AA disease severity scale, anchored in the extent of hair loss, captures key features commonly used by AA experts in clinical practice. This scale will better aid clinicians in appropriately assessing severity in patients with this common disease., Competing Interests: Conflicts of interest Dr King reports serving on advisory boards and/or is a consultant and/or is a clinical trial investigator for Aclaris Therapeutics Inc, Almirall, Arena Pharmaceuticals, Bioniz Therapeutics, Bristol-Meyers Squibb, Concert Pharmaceuticals Inc, Dermavant Sciences Inc, Eli Lilly and Company, Incyte Corp, Pfizer Inc, TWi Biotechnology Inc, and Viela Bio and is on speaker bureaus for Pfizer Inc, Regeneron, and Sanofi Genzyme. Dr Mesinkovska reports receiving honoraria and/or fees from Eli Lilly and Company outside the submitted work, serving as chief scientific officer for the National Alopecia Areata Foundation, and serves on advisory boards for Arena Pharmaceuticals, Concert Pharmaceuticals, Eli Lilly and Company, and Nutrafol. Dr Craiglow reports receiving honoraria and/or fees from Eli Lilly and Company outside the submitted work, Aclaris Therapeutics Inc, Arena Pharmaceuticals Inc, Pfizer Inc, Regeneron Pharmaceuticals Inc, and Sanofi Genzyme. Dr Kindred reports receiving honoraria and/or fees from Eli Lilly and Company outside the submitted work, serves on advisory boards for Eli Lilly and Company and UCB, is a speaker for Selphyl and Aerolase, and is the Chair of the National Medical Association, Dermatology Section. Dr Ko serves on advisory boards and is a consultant and clinical investigator for Eli Lilly and Company, and he has served as a clinical investigator and/or consultant for AbbVie, Sanofi, Regeneron, Dermira, BMS, and Arena Pharmaceuticals and has received consulting fees from Eli Lilly and Company, Concert Pharmaceuticals, and Arena Pharmaceuticals outside the submitted work. Dr McMichael reports receiving honoraria and/or fees from Eli Lilly and Company outside the submitted work, serving as a consultant for Eli Lilly and Company, and Pfizer Inc, and as a researcher for Concert Pharmaceuticals. Dr Shapiro reports serving as a consultant for Eli Lilly and Company, as a consultant and investigator for Pfizer Inc, and has received honoraria from Pfizer Inc, Eli Lilly and Company, Applied Biology, and DS Laboratories outside the submitted work, and served as an investigator for RegenLab and received stock options for Eirion and Replicel Life Sciences. Dr Mirmirani reports acting as a Principal Investigator, clinical trials for Concert Pharmaceuticals, Eli Lilly and Company, and Pfizer Inc, is a compensated consultant/advisory board member for Eli Lilly and Company, and a consultant for DS Laboratories, Monat Global, Almirall, Thirty Madison, Leo Pharmaceuticals, Bristol Myers Squibb, P&G. Dr Hordinsky reports being an investigator for alopecia areata clinical trials sponsored by Pfizer Inc, Eli Lilly and Company, and Concert Pharmaceuticals and being a consultant for CASSIOPEA S.p.A., and the immediate past president of the American Hair Research Society. Dr Huang reports royalty payments to her institution from Pfizer for licensing of the ALTO tool and consulting fees from Concert Pharmaceuticals, Pfizer Inc, and Eli Lilly and Company. Dr Castelo-Soccio reports receiving fees for alopecia-related presentations, participating in a scientific advisory panel for Pfizer Inc and on the medical advisory board for the National Alopecia Areata Foundation. Dr Bergfeld reports personal and other fees from Eli Lilly and Company, Chairing the International Federation of Hair Research, and being a former president of the American Hair Research Society. Dr Paller reports being an investigator for AbbVie, AnaptysBio, Eli Lilly, Incyte, KrystalBio, Janssen, and Regeneron, on a data safety monitoring board for AbbVie and Galderma, and a consultant with honorarium for AbbVie, Abeona, Almirall, Amagma, Anaptysbio, Arena, Bausch, Bristol Myer Squibb, Dermavant, Dermira, Eli Lilly, Exicure, Forte, Leo, Lifemax, Phoenix, Pierre Fabre, Pfizer, Rapt, Regeneron, Sanofi, Sol-Gel, UCB, and Venthera. Dr Mackay-Wiggan reports receiving honoraria and/or fees from Eli Lilly and Company, being a consultant for Pfizer Inc, Eli Lilly and Company, and Concert Pharmaceuticals, and participating in an advisory board for Eli Lilly and Company, and receipt of site-related equipment and consumables from Pfizer Inc and Concert Pharmaceuticals. Dr Glashofer reports serving as a consultant for Eli Lilly and Company. Dr Piliang reports receiving grants/contract for study sites from Pfizer Inc, and Eli Lilly and Company. Dr Lo Sicco reports receiving a grant from Pfizer Education for alopecia areata-related work, and being a RegenLab, and Pfizer Inc. Dr Cassella reports being an employee and stockholder of Concert Pharmaceuticals. Author Koenigsberg reports being an employee and stockholder of Concert Pharmaceuticals. Dr Ghorayeb reports being an employee and stockholder of Janssen Global Services, LLC. Dr Fakharzadeh reports being a current employee and stockholder of Janssen Global Services, LLC. Dr Napatalung reports being a current employee and stockholder of Pfizer Inc. Author Gandhi reports being an employee and stockholder of Pfizer Inc. Dr DeLozier reports being an employee and stockholder at Eli Lilly and Company. Dr Nunes reports being an employee and stockholder at Eli Lilly and Company during manuscript development and being a current employee and stockholder at Janssen Global Services, LLC. Dr Senna reports clinical trial funding and SAB consulting fees from Eli Lilly and Company, during the conduction of the study and reported clinical trial funding from Concert Pharmaceuticals and SAB consulting fees from Pfizer Inc. Drs Tosti, Aguh, and Ahluwalia do not have conflicts of interest to disclose., (Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Alopecia areata (AA) is an autoimmune form of hair loss with limited treatments., Objective: To evaluate the efficacy and safety of the Janus kinase inhibitors ritlecitinib and brepocitinib in patients who have AA with ≥ 50% scalp hair loss., Methods: Patients were randomized to once-daily ritlecitinib, brepocitinib, or placebo. The primary efficacy endpoint was a 24-week change from baseline in the Severity of Alopecia Tool (SALT) score. The key secondary efficacy endpoint was the proportion of patients achieving 30% improvement in SALT score (SALT 30 )., Results: The ritlecitinib, brepocitinib, and placebo groups included 48, 47, and 47 patients, respectively. At week 24, least-squares mean difference from placebo in SALT score change from baseline was 31.1 (95% confidence interval [CI], 18.8-43.5) for ritlecitinib and 49.2 (95% CI, 36.6-61.7) for brepocitinib (P < .0001 for both comparisons with placebo). SALT 30 was achieved by 50% (90% CI, 38%-62%) of patients receiving ritlecitinib, 64% (90% CI, 51%-75%) receiving brepocitinib, and 2% (90% CI, 0%-9%) receiving placebo. Two patients experienced a serious adverse event (rhabdomyolysis) in the brepocitinib group only., Limitations: Only a single-dosage regimen of each study drug was included., Conclusion: Treatment with ritlecitinib or brepocitinib for 24 weeks was efficacious and generally well tolerated., Competing Interests: Conflicts of interest Dr King is a clinical trial investigator for Pfizer, Concert Pharmaceuticals, and Eli Lilly and Company and has received honoraria and/or consulting fees from Aclaris Therapeutics, Arena Pharmaceuticals, Bristol-Meyers Squibb, Celgene, Concert Pharmaceuticals, Dermavant Sciences, Eli Lilly and Company, Pfizer, Regeneron, and Sanofi Genzyme. Dr Guttman-Yassky has received institutional grants from AbbVie, Celgene, Eli Lilly, Janssen, Dermavant, DS Biopharma, Novartis, Pfizer, Regeneron, Glenmark, Galderma, Asana Biosciences, Innovaderm, Dermira, LEO Pharma, Novan, Kyowa Kirin, Concert, Union Therapeutics, and Ralexar and is a consultant for Sanofi, Regeneron, Celgene, Dermira, Galderma, Glenmark, Novartis, Pfizer, LEO Pharma, AbbVie, Eli Lilly, Kyowa Kirin, Mitsubishi Tanabe, Asana Biosciences, Union Therapeutics, Allergan, Amgen, Concert, DS Biopharma, EMD Serono, Escalier, and Flx Bio. Drs Peeva and Banerjee are employees and stockholders of Pfizer. Dr Sinclair has provided professional services for Novartis, Merck & Co, Janssen, Samson Clinical, Pfizer, Eli Lilly and Company, Arena, Demira, AstraZeneca, Sanofi, AbbVie, Galderma, Principia, Reistone Pharma, Aclaris, and Sun Pharma. Dr Zhu is an employee and stockholder of Pfizer. Dr Cox is a paid consultant to Pfizer. Dr Craiglow has participated on advisory boards, received honoraria and consulting fees from Aclaris, Arena Pharmaceuticals, and Pfizer, and participated on the speaker's bureau for Regeneron and Sanofi-Genzyme. Drs Chen and Zhang were employees of Pfizer at the time the study was conducted. Drs Banfield, Page, and Vincent are employees and stockholders of Pfizer. Dr Pavel has no conflicts of interest to declare., (Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: We previously reported the Alopecia Areata Consensus of Experts study, which presented results of an international expert opinion on treatments for alopecia areata., Objective: To report the results of the Alopecia Areata Consensus of Experts international expert opinion on diagnosis and laboratory evaluation for alopecia areata., Methods: Fifty hair experts from 5 continents were invited to participate in a 3-round Delphi process. Consensus threshold was set at greater than or equal to 66%., Results: Of 148 questions, expert consensus was achieved in 82 (55%). Round 1 consensus was achieved in 10 of 148 questions (7%). Round 2 achieved consensus in 47 of 77 questions (61%). The final face-to-face achieved consensus in 25 of 32 questions (78%). Consensus was greatest for laboratory evaluation (12 of 14 questions [86%]), followed by diagnosis (11 of 14 questions [79%]) of alopecia areata. Overall, etiopathogenesis achieved the least category consensus (31 of 68 questions [46%])., Limitations: The study had low representation from Africa, South America, and Asia., Conclusion: There is expert consensus on aspects of epidemiology, etiopathogenesis, clinical features, diagnosis, laboratory evaluation, and prognostic indicators of alopecia areata. The study also highlights areas where future clinical research could be directed to address unresolved hypotheses in alopecia areata patient care., (Copyright © 2020 American Academy of Dermatology, Inc. All rights reserved.)

Importance: A recent expert consensus exercise emphasized the importance of developing a global network of patient registries for alopecia areata to redress the paucity of comparable, real-world data regarding the effectiveness and safety of existing and emerging therapies for alopecia areata., Objective: To generate core domains and domain items for a global network of alopecia areata patient registries., Evidence Review: Sixty-six participants, representing physicians, patient organizations, scientists, the pharmaceutical industry, and pharmacoeconomic experts, participated in a 3-round eDelphi process, culminating in a face-to-face meeting at the World Congress of Dermatology, Milan, Italy, June 14, 2019., Findings: Ninety-two core data items, across 25 domains, achieved consensus agreement. Twenty further noncore items were retained to facilitate data harmonization in centers that wish to record them. Broad representation across multiple stakeholder groups was sought; however, the opinion of physicians was overrepresented., Conclusions and Relevance: This study identifies the domains and domain items required to develop a global network of alopecia areata registries. These domains will facilitate a standardized approach that will enable the recording of a comprehensive, comparable data set required to oversee the introduction of new therapies and harness real-world evidence from existing therapies at a time when the alopecia areata treatment paradigm is being radically and positively disrupted. Reuse of similar, existing frameworks in atopic dermatitis, produced by the Treatment of Atopic Eczema (TREAT) Registry Taskforce, increases the potential to reuse existing resources, creates opportunities for comparison of data across dermatology subspecialty disease areas, and supports the concept of data harmonization.

Background: A systematic review failed to identify any systemic therapy used in alopecia areata (AA) where use is supported by robust evidence from high-quality randomized controlled trials., Objective: To produce an international consensus statement on the use and utility of various treatments for AA., Methods: Fifty hair experts from 5 continents were invited to participate in a 3-round Delphi process. Agreement of 66% or greater was considered consensus., Results: In the first round, consensus was achieved in 22 of 423 (5%) questions. After a face-to-face meeting in round 3, overall, consensus was achieved for only 130 (33%) treatment-specific questions. There was greater consensus for intralesional treatment of AA (19 [68%]) followed by topical treatment (25 [43%]). Consensus was achieved in 45 (36%) questions pertaining to systemic therapies in AA. The categories with the least consensus were phototherapy and nonprescription therapies., Limitations: The study included a comprehensive list of systemic treatments for AA but not all treatments used., Conclusion: Despite divergent opinions among experts, consensus was achieved on a number of pertinent questions. The concluding statement also highlights areas where expert consensus is lacking and where an international patient registry could enable further research., (Copyright © 2020 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Mutations in several lipid synthetic enzymes that block fatty acid and ceramide production produce autosomal recessive congenital ichthyoses (ARCIs) and associated abnormalities in permeability barrier homeostasis. However, the basis for the phenotype in patients with NIPAL4 (ichthyin) mutations (among the most prevalent ARCIs) remains unknown. Barrier function was abnormal in an index patient and in canines with homozygous NIPAL4 mutations, attributable to extensive membrane stripping, likely from detergent effects of nonesterified free fatty acid. Cytotoxicity compromised not only lamellar body secretion but also formation of the corneocyte lipid envelope (CLE) and attenuation of the cornified envelope (CE), consistent with a previously unrecognized, scaffold function of the CLE. Together, these abnormalities result in failure to form normal lamellar bilayers, accounting for the permeability barrier abnormality and clinical phenotype in NIPA-like domain-containing 4 (NIPAL4) deficiency. Thus, NIPAL4 deficiency represents another lipid synthetic ARCI that converges on the CLE (and CE), compromising their putative scaffold function. However, the clinical phenotype only partially improved after normalization of CLE and CE structure with topical ω-O-acylceramide because of ongoing accumulation of toxic metabolites, further evidence that proximal, cytotoxic metabolites contribute to disease pathogenesis., (Copyright © 2018 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.)

Goltz syndrome (focal dermal hypoplasia) is an X-linked dominant disorder that is classically associated with yellowish papules representing fat herniation (superficial adipocytes). We report a series of three cases, with clinicopathologic correlation of biopsies from Blaschkoid streaks. A range of histopathologic features, including some underreported findings (increased papillary dermal blood vessels, decreased thickness of the dermis, and adipocytes high in the dermis), are reproducible and can strongly point to the correct diagnosis of Goltz syndrome., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objectives: The goal of this study was to determine childhood clinical predictors of quality of life (QoL) in early adulthood in children with obsessive-compulsive disorder (OCD)., Methods: A longitudinal cohort study was conducted with 36 (out of 62 eligible) children with OCD, interviewed once at childhood baseline (mean age 12.1 ± 2.1, range 8.0-15.8), and again in early adulthood after an average follow-up interval of 9 years. QoL was measured in adulthood with the longitudinal interval follow-up evaluation range of impaired functioning tool (LIFE-RIFT)., Results: Forty-two percent of children experienced a remission of OCD symptoms by early adulthood. OCD appeared to most strongly impair the interpersonal relationships and work domains of QoL. QoL and severity of OCD and anxiety symptoms were significantly associated in early adulthood. Primary hoarding symptoms in childhood predicted poor QoL in adulthood. Increased symptoms in the forbidden thoughts dimension in both childhood and adulthood were associated with improved adulthood QoL., Conclusions: Children for whom OCD symptoms remitted by adulthood showed no evidence of residual impairment in QoL, whereas children whose OCD symptoms failed to remit by adulthood showed at most mild impairment in QoL. Hoarding symptoms in childhood appear to portend not only the persistence of OCD symptoms but also poorer QoL in early adulthood.

Background: Cardioversion and defibrillation have become widely used techniques aimed at restoring normal sinus rhythm in patients with cardiac arrhythmias. Following the procedure, cutaneous lesions are often seen at the site of the electrodes, but little has been reported regarding the evolution of such lesions over time., Observations: Two patients presented with unusual, well-defined rectangular eruptions on the left back, and both reported a history of having undergone electrical cardioversion or defibrillation several years previously. The histologic characteristics of each lesion were distinct, and the management was symptomatic, with most of the relief coming from the recognition that the eruption was actually a self-limited manifestation of cardioversion and defibrillation., Conclusions: The clinical cases and corresponding histologic findings represent possible long-term sequelae of electrical cardioversion or defibrillation. They are presented in order to enhance the diagnostic acumen of dermatologists and to avoid potential misdiagnosis.

We report a case of primary cutaneous aspergillosis in an immunocompetent child that responded rapidly to oral voriconazole therapy. Voriconazole may be considered as a treatment option for pediatric patients with primary cutaneous aspergillosis.