Amy S. Paller's research while affiliated with Ann & Robert H. Lurie Children's Hospital of Chicago and other places

Publications (803)

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Background To report baseline characteristics, patient reported outcomes and treatment of children with Juvenile Dermatomyositis (JDM) in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Methods Children newly diagnosed with JDM were enrolled in the CARRA Registry from 41 pediatric rheumatology centers. Baseline patient...
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Background Upadacitinib is a selective, reversible, Janus kinase (JAK) inhibitor with established efficacy in moderate-to-severe atopic dermatitis (AD). Objective Evaluate the safety of upadacitinib in patients with moderate-to-severe AD. Methods Integrated safety data from the 16-week placebo-controlled periods of 1 phase 2b and 3 ongoing phase...
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Background Current systemic treatments for children younger than 6 years with moderate-to-severe atopic dermatitis that is uncontrolled with topical therapies might have suboptimal efficacy and safety. Dupilumab is approved for older children and adults with atopic dermatitis and for other type 2 inflammatory conditions. We aimed to evaluate effica...
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Characterize short‐term kidney status and describe variation in early care utilization in a multi‐center cohort of patients with childhood‐onset systemic lupus erythematosus (cSLE) and nephritis. We analyzed previously collected prospective data from North American cSLE patients with kidney biopsy‐proven nephritis enrolled in the Childhood Arthriti...
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Background Standardized assessments for dystrophic epidermolysis bullosa (DEB) are needed. This prospective, multicenter, 4-week, observational study was designed to evaluate DEB assessments for suitability as clinical trial endpoints. Methods Patients with confirmed DEB diagnosis and ≥ 5 measurable wounds were included. The primary outcome was ch...
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Importance: Molluscum contagiosum (MC) is a highly contagious skin condition. Lesions may persist for months to years, and no US Food and Drug Administration-approved medications are currently available in the US. Objective: To assess the efficacy and safety of berdazimer gel, 10.3%, a novel topical nitric oxide-releasing medication, in the trea...
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Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disease characterized by pruritic erythematous scaly plaques distributed along the lines of Blaschko. Two cases of ILVEN with CARD14 mutations and one case with a GJA1 mutation have been previously reported. To elucidate the genetic cause of a cohort of patients diagnosed based on...
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“How to” skin care manuals assist health care professionals caring for hospitalized patients with epidermolysis bullosa and other disorders. Manuals created by Epidermolysis Bullosa Clinical Research Consortium sites were collected and analyzed. Analysis of manuals revealed variable content. Creating a unified, optimized “how” to manual would benef...
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Atopic dermatitis (AD) is a chronic, heterogenous, inflammatory skin disorder associated with a high skin‐related health burden, typically starting in childhood and often persisting into adulthood. AD is characterized by a wide range of clinical phenotypes, reflecting multiple underlying pathophysiological mechanisms and interactions between geneti...
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Background: For adolescent patients (aged ≥ 12 to < 18 years) with uncontrolled moderate-to-severe atopic dermatitis (AD), 16 weeks of treatment with dupilumab resulted in substantial clinical benefit compared with placebo, with an acceptable safety profile. However, long-term data on the approved dose regimens of dupilumab in adolescents with AD...
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Background: The ichthyoses are rare genetic keratinizing disorders that share the characteristics of an impaired epidermal barrier and increased risk of microbial infections. Although ichthyotic diseases share a Th17 immune signature, including increased expression of antimicrobial peptides, the skin microbiota of ichthyoses is virtually unexplore...
Preprint
BACKGROUND Atopic dermatitis (AD) is a pruritic chronic condition associated with significant sleep disturbance, inattention, and sometimes behavioral problems. Enhancing resiliency in children with AD may promote coping strategies to improve quality of life. One strategy for strengthening resiliency is via positive psychology. OBJECTIVE Our objec...
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Background Atopic dermatitis is a pruritic chronic condition associated with significant sleep disturbance, inattention, and sometimes behavioral problems. Enhancing resiliency in children with atopic dermatitis may promote coping strategies to improve quality of life. Positive psychology is one strategy that can be used to strengthen resiliency....
Article
Importance: About 1% of children and adolescents worldwide are affected by plaque psoriasis. Objective: To evaluate the long-term efficacy and safety of ixekizumab for pediatric patients with moderate to severe psoriasis. Design, setting, and participants: This multicenter randomized clinical trial (IXORA-PEDS) evaluated pediatric patients wit...
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Background: The incidence of systemic lupus in children with discoid lupus is unknown. Objective: This study assessed the baseline characteristics of patients with pediatric discoid lupus erythematosus (pDLE). Methods: Medical records at 17 sites were reviewed for pediatric dermatology and rheumatology patients with discoid lupus erythematosus...
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Preliminary work suggested upregulation of inflammatory pathways in patients with common forms of ichthyosis. However, a comprehensive characterization of skin from various ichthyosis subtypes is unavailable, precluding development of targeted treatments. Thus, we sought to characterize the immune and barrier profiles of common and subtype-specific...
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Background: The validated Investigator Global Assessment for Atopic Dermatitis (vIGA-AD™) is a standardized severity assessment for use in clinical trials and registries for atopic dermatitis (AD). Objectives: To investigate the reliability, validity, responsiveness, and within-patient meaningful change of the vIGA-AD. Methods: Data were from...
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Background Social determinants of health (SDH) greatly influence outcomes during the first year of treatment in rheumatoid arthritis, a disease similar to polyarticular juvenile idiopathic arthritis (pJIA). We investigated the correlation of community poverty level and other SDH with the persistence of moderate to severe disease activity and functi...
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Objectives: To identify pediatric patient-reported outcomes (PROs) that are associated with chronic conditions and to evaluate the effects of chronic disease activity on PROs. Study design: Participants 8-24 years-old and their parents were enrolled into 14 studies that evaluated PROMIS® PROs across 10 chronic conditions--asthma, atopic dermatit...
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Objective Provide a review of atopic dermatitis management, focusing on optimizing topical therapy, creating a stepwise approach for treatment plans, and guide when to start systemic therapy. Data Sources PubMed search of English-language articles regarding atopic dermatitis in all ages. Study Selection Articles on the subject matter were selecte...
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Alopecia areata/AA is an autoimmune cause of nonscarring hair loss. The pathogenesis of AA involves many immune axes, including Th1/Th2 pathways. Delgocitinib is a pan-Janus kinase/JAK inhibitor that broadly blocks pro-inflammatory cytokines and has been effective in other inflammatory skin conditions. Recent human studies/reports have shown that u...
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Importance Treatment of congenital ichthyoses primarily focuses on reversing skin scaling and is not pathogenesis based. Recent studies showed Th17 immune skewing, as in psoriasis, across the spectrum of ichthyosis, suggesting that targeting this pathway might broadly reduce disease severity. Objective To determine whether secukinumab, an IL-17A i...
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Importance: A comprehensive, user-friendly system to assess global ichthyosis disease burden is imperative to improving the care of patients with ichthyosis, identifying appropriate participants for clinical trials, and quantifying treatment outcomes. To our knowledge, there is currently no validated scale to objectively and systematically measure...
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Psoriasis vulgaris is an inflammatory skin disease that affects 2‐3% of the population worldwide. One of the major challenges in discovering novel therapies is the poor translatability of animal models to human disease. Therefore, it is imperative to develop human preclinical models of psoriasis that are amenable to pharmacological intervention. He...
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Background/objective: Patients with moderate-to-severe atopic dermatitis (AD) have increased risk of cutaneous and extracutaneous infections. Dupilumab has previously been associated with reduced risk of serious/severe infections and non-herpetic skin infections in adults with moderate-to-severe AD. This analysis assessed infection rates with dupi...
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Background Studies found associations between atopic dermatitis (AD) and many comorbidities. Objective To appraise evidence of the association between AD and comorbidities among adults. Methods Our multidisciplinary work group conducted a systematic review of the association between AD and selected comorbidities. We applied the GRADE for prognosi...
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Objective To determine the dose-response relationship of tumor necrosis factor (TNF) inhibition in the treatment of juvenile idiopathic arthritis (JIA). Methods Participants of the Childhood Arthritis and Rheumatology Research Alliance Registry were eligible for inclusion in the analyses if they started TNF inhibition for JIA. The primary treatmen...
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This study is a retrospective analysis using data collected from the Adelphi Paediatric Psoriasis Disease-Specific Programme cross-sectional survey. Despite being treated for their psoriasis, a substantial proportion of paediatric patients presented with moderate (18.3%) or severe (1.3%) disease at sampling; 42.9% and 92.0% had a body surface area...
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Background/Objectives Older children with atopic dermatitis (AD) suffer from poor sleep and attention problems. However, until recently, the dearth of developmentally sensitive assessment tools impeded characterization in younger children. We aimed to characterize sleep and attention problems in young children with AD and identify modifiable factor...
Article
Introduction Une barrière cutanée saine favorise la croissance des bactéries commensales, qui protègent l’hôte contre les bactéries pathogènes et les facteurs de virulence. Des études récentes sur la dermatite atopique (DA) ont mis en évidence un manque de diversité microbienne dans la peau lésionnelle et non lésionnelle. La dérégulation de la micr...
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Background The impact of social determinants of health on children with polyarticular juvenile idiopathic arthritis (pJIA) is poorly understood. Prompt initiation of treatment for pJIA is important to prevent disease morbidity; however, a potential barrier to early treatment of pJIAs is delayed presentation to a pediatric rheumatologist. We examine...
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Background We aimed to characterize etanercept (ETN) use in juvenile idiopathic arthritis (JIA) patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. Methods The CARRA Registry is a convenience cohort of patients with paediatric onset rheumatic diseases, including JIA. JIA patients treated with ETN for w...
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Background Biologics represent a promising treatment for children with moderate-to-severe psoriasis. Randomized control trials (RCTs) have been published evaluating different biologics in children with psoriasis, but no summative review exists. Objective To summarize data from existing RCTs to assess the efficacy and safety of biologics in the man...
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Importance Ichthyoses are clinically and genetically heterogeneous disorders characterized by scaly skin. Despite decades of investigation identifying pathogenic variants in more than 50 genes, clear genotype-phenotype associations have been difficult to establish. Objective To expand the genotypic and phenotypic spectra of ichthyosis and delineat...
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Introduction Pruritus is a common symptom across various dermatologic conditions, with a negative impact on quality of life. Devices to quantify itch objectively primarily use scratch as a proxy. This review compares and evaluates the performance of technologies aimed at objectively measuring scratch behavior. Methods Articles identified from lite...
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Background Accurate assessment of atopic dermatitis (AD) severity is critical when initiating and monitoring therapy. Use of existing research tools such as the Eczema Area and Severity Index (EASI) and the SCORing Atopic Dermatitis (SCORAD) is complex and time-consuming in clinical practice. A previous analysis found the product of validated Inves...
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Background/objectives: Psoriasiform eruptions after initiation of dupilumab have been previously described in adults. This report details the risk of developing or unmasking psoriasiform eruptions after initiation of dupilumab in children. Methods: Records of patients ≤18 years of age with atopic dermatitis who developed psoriasiform dermatitis...
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Background: Accurate diagnosis of epidermolysis bullosa (EB) has significant implications for prognosis, management, and genetic counseling. Objective: To describe diagnostic testing patterns and assess diagnostic concordance of transmission electron microscopy (TEM), immunofluorescence mapping (IFM), and genetic analysis for EB. Methods: A re...
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Background Patients with atopic dermatitis (AD) are considered at increased risk of developing other type 2 inflammatory diseases. However, real-world evidence based on large US commercially insured pediatric populations is scarce. Objective To use a large US claims database (IBM® MarketScan® 2013-2017) to assess prevalence and incidence of type 2...
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Itch compromises quality of life, but most itch assessments focus only on itch intensity. We aimed to develop and validate a comprehensive PROMIS (Patient Reported Outcomes Measurement Information System®) pediatric measure for itch symptoms and itch impact, defined as the effect specifically of itch on physical, mental, and social health, all of w...