Publications by authors named "Heidi Jacobe"

65 Publications

Correction to: Single-cell transcriptome conservation in a comparative analysis of fresh and cryopreserved human skin tissue: pilot in localized scleroderma.

Arthritis Res Ther 2021 Apr 6;23(1):101. Epub 2021 Apr 6.

Division of Rheumatology, Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13075-021-02490-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8022423PMC
April 2021

Community-based melanoma screening: A pilot study for the development of an educational curriculum for nonphysician providers.

J Am Acad Dermatol 2021 Mar 29. Epub 2021 Mar 29.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2021.02.037DOI Listing
March 2021

Body site distribution of pediatric-onset morphea and association with extracutaneous manifestations.

J Am Acad Dermatol 2021 Mar 6. Epub 2021 Mar 6.

Departments of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.

Background: The distribution of pediatric-onset morphea and site-based likelihood for extracutaneous complications has not been well characterized.

Objective: To characterize the lesional distribution of pediatric-onset morphea and to determine the sites with the highest association of extracutaneous manifestations.

Methods: A retrospective cross-sectional study was performed. Using clinical photographs, morphea lesions were mapped onto body diagrams using customized software.

Results: A total of 823 patients with 2522 lesions were included. Lesions were more frequent on the superior (vs inferior) anterior aspect of the head and extensor (vs flexor) extremities. Linear morphea lesions were more likely on the head and neck, whereas plaque and generalized morphea lesions were more likely on the trunk. Musculoskeletal complications were more likely with lesions on the extensor (vs flexor) extremity (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.4), whereas neurologic manifestations were more likely with lesions on the anterior (vs posterior) (OR, 2.8; 95% CI, 1.7-4.6) and superior (vs inferior) aspect of the head (OR, 2.3; 95% CI, 1.6-3.4).

Limitations: Retrospective nature and the inclusion of only patients with clinical photographs.

Conclusion: The distribution of pediatric-onset morphea is not random and varies with body site and within individual body sites. The risk stratification of extracutaneous manifestations by body site may inform decisions about screening for extracutaneous manifestations, although prospective studies are needed.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2021.03.017DOI Listing
March 2021

Melanoma toolkit for early detection (MTED) for primary care providers: A pilot study.

Pigment Cell Melanoma Res 2021 Feb 26. Epub 2021 Feb 26.

Oregon Health and Science University, Department of Dermatology.

Introduction: Primary care providers have greater access to patients despite often lacking the appropriate training or time to implement effective skin cancer screenings in their busy practices. Through collaborative efforts with Oregon's War on Melanoma public health campaign and other primary care trainings, we created "Melanoma Toolkit for Early Detection" (MTED): a training curriculum and resource repository for primary care providers.

Methods And Results: MTED consisted of three self-paced modules. Each participant completed a pre- and post-test consisting of demographic, confidence, and image identification questions. A subsequent optional 6-month follow-up image identification test was also provided. Of the 96 participants, 40 completed all the modules, the pre, and post-test. On average, scores increased by 6.0 (95% CI: 3.5 to 8.6) percentage points (P<0.001, paired t-test). The percent of participants reporting confidence with melanoma identification increased significantly from the pre- (23.3%) to the post-test (67.4%), an increase of 44.2 (95% CI: 29.3 to 59.0,) percentage points (P<0.001, McNemar's test).

Discussion: This study shows that an online curriculum such as MTED has the potential to increase PCPs confidence and knowledge. This could subsequently lead to improved early melanoma diagnosis by primary care providers.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/pcmr.12968DOI Listing
February 2021

An Evaluation of the Performance of Current Morphea Subtype Classifications.

JAMA Dermatol 2021 Feb 17. Epub 2021 Feb 17.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas.

Importance: Numerous classification systems for morphea subtypes exist, but none have been systematically evaluated for their ability to categorize patients with morphea into demographically and clinically coherent groups. Although some subtypes, such as linear morphea, are present across all the classification schemes, others list unique subtypes. This creates confusion among investigators and practitioners and impairs accurate categorization of patients for study and clinical evaluation.

Objective: To evaluate how frequently the commonly used morphea classification systems categorize patients with morphea into clinically relevant subtypes using cross-sectional analysis of 2 large patient cohorts.

Design, Setting, And Participants: This cross-sectional study comprised 944 adults and children from 2 prospective cohorts-the Morphea in Adults and Children at the University of Texas Southwestern Medical Center (Dallas, Texas), which enrolled participants from July 20, 2007, to September 21, 2018, and the National Registry for Childhood-Onset Scleroderma at the University of Pittsburgh (Pittsburgh, Pennsylvania), which enrolled participants from October 23, 2002, to November 13, 2018.

Main Outcomes And Measures: Patient demographic characteristics, morphea subtype, quality-of-life measures, disease activity, and damage as measured by Localized Scleroderma Cutaneous Assessment Tool scores during initial visits.

Results: A total of 944 participants (444 patients with adult-onset morphea and 500 patients with pediatric-onset morphea; 741 female participants [78%]; median age at onset, 16 years [interquartile range, 8-44 years]) were included in this study. Most participants were White (723 [77%]) and had the linear (474 [50%]) or generalized subtype of morphea (244 [26%]). With the use of the previously published Padua criteria, most patients were classified to have linear morphea (474 [50%]), followed by generalized morphea (244 [26%]), plaque morphea (141 [15%]), mixed morphea (38 [4%]), and pansclerotic morphea (3 [0.3%]). Overall, the Padua criteria successfully classified 900 patients (95%) in comparison with the Peterson criteria (533 [56%]) and the European Dermatology Forum classification (487 [52%]).

Conclusions And Relevance: In this cross-sectional study of morphea subtype classification systems, the Padua criteria performed best in classifying patients into subgroups with cohesive demographic and clinical features, supporting its widespread use. However, they have ambiguities that might lead to misclassification, particularly in terms of generalized and pansclerotic morphea and descriptors such as morphea profunda. Consensus-based approaches are needed to address these ambiguities and develop a unified classification scheme.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1001/jamadermatol.2020.5809DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7890441PMC
February 2021

Morphea patients with mucocutaneous involvement: A cross sectional study from the Morphea in Adults and Children (MAC) cohort.

J Am Acad Dermatol 2020 Nov 26. Epub 2020 Nov 26.

Department of Dermatology, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, Texas 75390-9069. Electronic address:

Background: Demographic and clinical findings of patients with mucocutaneous morphea have not been well characterized.

Objective: To determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions enrolled in the Morphea in Adults and Children Cohort (MAC).

Methods: Cross-sectional study of 735 patients in the MAC Cohort from 2007 to 2018.

Results: 4.6% of linear morphea patients had oral involvement versus 2.4% among the entire cohort while 10.3% of generalized morphea patients had genital involvement versus 3.7% among the entire cohort. Those with genital lesions had an older age of disease onset than oral morphea (57 versus 11.5 years old; p<0.001) and more frequent extragenital lichen sclerosus atrophicus (LSA) (59.2% versus 5.6%; p=0.004).

Limitations: Selection bias and limited number of affected subjects.

Conclusion: Oral morphea lesions predominate in younger patients with facial linear morphea while genital lesions predominate in post-menopausal women with overlying extragenital LSA.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2020.10.093DOI Listing
November 2020

Single-cell transcriptome conservation in a comparative analysis of fresh and cryopreserved human skin tissue: pilot in localized scleroderma.

Arthritis Res Ther 2020 11 9;22(1):263. Epub 2020 Nov 9.

Division of Rheumatology, Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA.

Background: The purpose of this study was to assess variability in cell composition and cell-specific gene expression in the skin of patients with localized scleroderma (LS) utilizing CryoStor® CS10 in comparison to RPMI to produce adequate preservation of tissue samples and cell types of interest for use in large-scale multi-institutional collaborations studying localized scleroderma and other skin disorders.

Methods: We performed single-cell RNA sequencing on paired skin biopsy specimens from 3 patients with LS. Each patient with one sample cryopreserved in CryoStor® CS10 and one fresh in RPMI media using 10× Genomics sequencing.

Results: Levels of cell viability and yield were comparable between CryoStor® CS10 (frozen) and RPMI (fresh) preserved cells. Furthermore, gene expression between preservation methods was collectively significantly correlated and conserved across all 18 identified cell cluster populations.

Conclusion: Comparable cell population and transcript expression yields between CryoStor® CS10 and RPMI preserved cells support the utilization of cryopreserved skin tissue in single-cell analysis. This suggests that employing standardized cryopreservation protocols for the skin tissue will help facilitate multi-site collaborations looking to identify mechanisms of disease in disorders characterized by cutaneous pathology.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13075-020-02343-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7654179PMC
November 2020

The importance of development standards for anchoring vignettes: an illustrative example from pediatric localized scleroderma quality of life.

Qual Life Res 2020 Dec 11;29(12):3263-3272. Epub 2020 Jul 11.

University of Pittsburgh School of Medicine, Pittsburgh, USA.

Purpose: Anchoring vignettes (AVs) are a promising measurement technique to reduce bias in patient-reported outcome (PRO) measures by helping researchers understand differences in how individuals and groups interpret response options. However, little attention has been paid to ensure quality development of AVs, and their performance has not been well assessed in pediatric populations. In this study, we explore the application of a rigorous development process for AVs based upon current standards for PROs, as well as feasibility of AVs when administered to children and adolescents.

Methods: We developed AVs using a rigorous, patient-centered mixed methods process including three phases: (1) development, (2) a pilot study, and (3) a field test. Our proposed process included the generation of a conceptual framework based on the PRO, the Localized Scleroderma Quality of Life Instrument, and numerous vignette-specific considerations. We qualitatively explored readability and comprehension of the AVs (pilot study) and then analyzed ranking patterns within vignette sets (field test).

Results: Four sets of four vignettes were developed. Revisions were suggested at each phase of development. The pilot study demonstrated that children ≥ 10 years had no trouble indicating understanding of the AVs. In the field test, although appropriate rankings of vignettes were generally demonstrated by participants, the percentage of tied rankings was higher than expected in this pediatric group.

Conclusions: This work supports the need for rigorous developmental standards for AVs, as each stage of development suggested revisions. Additionally, AVs showed initial promise for use with pediatric populations; general feasibility and understanding were supported.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s11136-020-02575-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7686110PMC
December 2020

Acute generalized exanthematous pustulosis induced by empiric hydroxychloroquine for presumed COVID-19.

Dermatol Ther 2020 Nov 8;33(6):e13834. Epub 2020 Jul 8.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/dth.13834DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7323399PMC
November 2020

Changes in Disease Activity and Damage Over Time in Patients With Morphea.

JAMA Dermatol 2020 05;156(5):513-520

Department of Dermatology, UT Southwestern Medical Center, Dallas, Texas.

Importance: Prospective studies of the disease course in patients with morphea are lacking, particularly those comparing adults and children.

Objective: To investigate the disease course in patients with morphea treated with standard-of-care therapy using validated clinical outcome measures.

Design, Setting, And Participants: Prospective cohort study of 130 adults and children from the Morphea in Adults and Children cohort with at least 2 years of clinical follow-up and Localized Scleroderma Cutaneous Assessment Tool scores recorded at each study visit. Study patients were seen at a tertiary referral center (UT Southwestern Medical Center, Dallas, Texas) from November 1, 2008, through April 1, 2016. The dates of analysis were May 2016 through July 2019.

Exposures: All patients received standard-of-care therapy.

Main Outcomes And Measures: Patterns in disease activity and recurrence were examined. The time to recurrence of morphea disease activity from the first visit with inactive disease was assessed using survival analysis with the log-rank test to compare differences between morphea subtypes.

Results: In total, 130 adults and children (663 study visits) were included in this study. The mean (SD) age of patients was 34.4 (23.8) years, and 101 of 130 (78%) were female. The mean (SD) follow-up was 4.3 (1.7) years. Fifty patients had at least 5 years of follow-up. Most patients were white individuals (96 of 130 [74%]) and had linear subtype (72 of 130 [55%]) or generalized subtype (40 of 130 [31%]). Overall, 13 of 30 (43%) with generalized subtype had recurrence of disease activity compared with 14 of 66 (21%) with linear subtype (hazard ratio, 3.28; 95% CI, 1.38-7.79). The median (interquartile range) time to first recurrence of disease activity after initial resolution of disease activity was 1.1 (0.8-1.9) years for generalized subtype and 2.3 (1.0-3.3) years for linear subtype. Of the 50 patients followed up for at least 5 years, 18 (36%) had recurrence of disease activity.

Conclusions And Relevance: Disease activity appeared to improve in most patients with morphea over 6 to 12 months using previously published treatment plans, underscoring their effectiveness. Sclerosis improved more slowly (over 2-5 years), often after discontinuation of treatment, but atrophy increased slightly as sclerosis subsided. Standard-of-care therapy appears to improve disease activity, which allows sclerosis to improve, and provides relative stability of other features of disease damage. A substantial number of patients, particularly those with generalized subtype, have a relapsing-remitting course over many years. Patients with morphea should be monitored for recurrent disease activity over extended periods.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1001/jamadermatol.2020.0034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7113826PMC
May 2020

Evaluation of the Effectiveness and Tolerability of Mycophenolate Mofetil and Mycophenolic Acid for the Treatment of Morphea.

JAMA Dermatol 2020 05;156(5):521-528

Department of Dermatology, Brigham and Women's Hospital, Boston, Massachusetts.

Importance: First-line systemic therapy for morphea includes methotrexate with or without systemic corticosteroids. When this regimen is ineffective, not tolerated, or contraindicated, a trial of mycophenolate mofetil (MMF) or mycophenolic acid (MPA)-referred to herein as mycophenolate-is recommended; however, evidence to support this recommendation remains weak.

Objective: To evaluate the effectiveness and tolerability of mycophenolate for the treatment of morphea.

Design, Setting, And Participants: A retrospective cohort study was conducted from January 1, 1999, to December 31, 2018, among 77 patients with morphea from 8 institutions who were treated with mycophenolate.

Main Outcomes And Measures: The primary outcome was morphea disease activity, severity, and response at 0, 3 to 6, and 9 to 12 months of mycophenolate treatment. A secondary outcome was whether mycophenolate was a well-tolerated treatment of morphea.

Results: There were 61 female patients (79%) and 16 male patients (21%) in the study, with a median age at disease onset of 36 years (interquartile range, 16-53 years) and median diagnostic delay of 8 months (interquartile range, 4-14 months). Generalized morphea (37 [48%]), pansclerotic morphea (12 [16%]), and linear morphea of the trunk and/or extremities (9 [12%]) were the most common subtypes of morphea identified. Forty-one patients (53%) had an associated functional impairment, and 49 patients (64%) had severe disease. Twelve patients received initial treatment with mycophenolate as monotherapy or combination therapy and 65 patients received mycophenolate after prior treatment was ineffective (50 of 65 [77%]) or poorly tolerated (21 of 65 [32%]). Treatments prior to mycophenolate included methotrexate (48 of 65 [74%]), systemic corticosteroids (42 of 65 [65%]), hydroxychloroquine (20 of 65 [31%]), and/or phototherapy (14 of 65 [22%]). After 3 to 6 months of mycophenolate treatment, 66 of 73 patients had stable (n = 22) or improved (n = 44) disease. After 9 to 12 months of treatment, 47 of 54 patients had stable (n = 14) or improved (n = 33) disease. Twenty-seven patients (35%) achieved disease remission at completion of the study. Treatments received in conjunction with mycophenolate were frequent. Mycophenolate was well tolerated. Gastrointestinal adverse effects were the most common (24 [31%]); cytopenia (3 [4%]) and infection (2 [3%]) occurred less frequently.

Conclusions And Relevance: This study suggests that mycophenolate is a well-tolerated and beneficial treatment of recalcitrant, severe morphea.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1001/jamadermatol.2020.0035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7113833PMC
May 2020

Career satisfaction of leaders in academic dermatology: Results from a national survey.

Int J Womens Dermatol 2020 Jan 25;6(1):25-29. Epub 2019 Oct 25.

CLEARS, Department of Dermatology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, United States.

Background: A positive correlation between leadership roles and job satisfaction has been noted in some areas of business. Since senior leaders in academic dermatology appear to be more satisfied than their junior colleagues, a similar relationship may be important in dermatology.

Objective: To determine if there is an association between leadership roles and career satisfaction of academic dermatologists.

Methods: A cross-sectional, anonymous survey was mailed to 1263 academic dermatologists across the US. Participants were questioned on demographics and career satisfaction. Academic rank and position was compared with career satisfaction.

Results: The leadership cohort was comprised of 140 (77%) men and 41 (23%) women (p < 0.01). Leaders were significantly more satisfied in their careers than non-leaders (65% versus 36%, p < 0.01), and were also less likely to leave academia. Factors related to career satisfaction included satisfaction with the promotion process (p < 0.01), presence of career development programs (p < 0.02), physician health (p < 0.01), and the ability to achieve balance in one's personal and professional lives (p = 0.01). Our analysis also demonstrated a gender gap within the leadership sector, with female leaders reporting less satisfaction overall with their career (44% versus 71%, p < 0.01), with the tenure/promotion process at their institutions (89% vs. 68%, respectively, p < 0.01), as well as their personal and professional balance (49% vs. 80%, p < 0.01) compared to their male leaders counterparts respectively. However, there was no difference in the likelihood of leaving academia between male and female leaders.

Conclusion: Academic leaders overall had higher career satisfaction than non-leaders, and were more likely to stay within academia. Despite this, patterns of gender disparities in the academic dermatology leadership persist with males outnumbering females in the leadership pool, and male leaders reporting higher levels of satisfaction compared to their female counterparts, as well as perceiving fewer challenges in finding balance between their personal and professional lives.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijwd.2019.09.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6997821PMC
January 2020

Utilizing UVA-1 Phototherapy.

Dermatol Clin 2020 Jan;38(1):79-90

University of Texas at Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-9069, USA. Electronic address:

There is a long history of utilization of phototherapy for treatment of skin conditions. Because of its longer wavelength, UVA1 phototherapy is able to penetrate into the dermis and subcutis. This depth of penetration, combined with its unique immunomodulating properties, makes UVA1 an effective treatment modality for many immune-mediated skin diseases. In some cases, it performs better than other types of phototherapy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.det.2019.08.011DOI Listing
January 2020

Hyaluronidase injections for treatment of symptomatic pansclerotic morphea-induced microstomia.

JAAD Case Rep 2019 Oct 25;5(10):871-873. Epub 2019 Sep 25.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jdcr.2019.08.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6804562PMC
October 2019

Immunopathogenesis of Pediatric Localized Scleroderma.

Front Immunol 2019 30;10:908. Epub 2019 Apr 30.

Division of Pediatric Rheumatology, Department of Rheumatology, Hospital for Special Surgery, New York, NY, United States.

Localized scleroderma (LS) is a complex disease characterized by a mixture of inflammation and fibrosis of the skin that, especially in the pediatric population, also affects extracutaneous tissues ranging from muscle to the central nervous system. Although developmental origins have been hypothesized, evidence points to LS as a systemic autoimmune disorder, as there is a strong correlation to family history of autoimmune disease, the presence of shared HLA types with rheumatoid arthritis, high frequency of auto-antibodies, and elevated circulating chemokines and cytokines associated with T-helper cell, IFNγ, and other inflammatory pathways. This inflammatory phenotype of the peripheral blood is reflected in the skin via microarray, RNA Sequencing and tissue staining. Research is underway to identify the key players in the pathogenesis of LS, but close approximation of inflammatory lymphocytic and macrophage infiltrate with collagen and fibroblasts deposition supports the notion that LS is a disease of inflammatory driven fibrosis. The immune system is dynamic and undergoes changes during childhood, and we speculate on how the unique features of the immune system in childhood could potentially contribute to some of the differences in LS between children and adults. Interestingly, the immune phenotype in pediatric LS resembles to some extent the healthy adult cellular phenotype, possibly supporting accelerated maturation of the immune system in LS. We discuss future directions in better understanding the pathophysiology of and how to better treat pediatric LS.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fimmu.2019.00908DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6503092PMC
September 2020

Linear morphea: Clinical characteristics, disease course, and treatment of the Morphea in Adults and Children cohort.

J Am Acad Dermatol 2019 Jun 17;80(6):1664-1670.e1. Epub 2019 Apr 17.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:

Background: Prospective, longitudinal studies examining the features of linear morphea are limited.

Objective: To utilize the Morphea in Adults and Children cohort to determine clinical characteristics, impact on life quality, and disease course of linear morphea in a prospective, longitudinal manner.

Methods: Characteristics of linear morphea versus other subtypes were compared in a cross-sectional manner. Next, linear morphea participants were examined in depth over a 3-year period.

Results: Linear morphea was the most common morphea subtype (50.1%, 291/581) in the cohort. Deep involvement was more common in linear (64.3%, 187/291) than other morphea subtypes. Linear morphea participants with deep involvement were more likely to have a limitation in range of motion (28.6%, 55/192) than those without (11.1%, 11/99, P < .001). Adult-onset disease occurred in 32.6% (95/291) of those with linear morphea. Frequency of deep involvement was similar between pediatric (66.8%, 131/196) and adult-onset linear morphea (58.9%, 56/95, P = .19). Quality of life and disease activity scores improved over time, while damage stabilized with treatment.

Limitations: Results of the study are associative, and the University of Texas Southwestern Medical Center is a tertiary referral center.

Conclusion: A substantial number of linear morphea patients have adult-onset disease. In all age groups, linear morphea with deep involvement was associated with functional limitations.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2019.01.050DOI Listing
June 2019

New Features for Measuring Disease Activity in Pediatric Localized Scleroderma.

J Rheumatol 2018 12 15;45(12):1680-1688. Epub 2018 Sep 15.

From the Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Department of Pediatrics, Hackensack; Stevens Institute of Technology, Hoboken, New Jersey, USA; Hospital for Sick Children, Toronto, Ontario, Canada; Texas Scottish Rite Hospital, University of Texas (UT) Southwestern, Dallas, Texas; The Ohio State University and Nationwide Children's Hospital, Columbus, Ohio; Duke University, Durham, North Carolina; Riley Hospital for Children at Indiana University Health, Indianapolis, Indiana; Rockefeller University, New York, New York, USA; Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany; Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.

Objective: To identify clinical features that define disease activity in pediatric localized scleroderma (LS), and determine their specificity and importance.

Methods: We conducted a multicenter prospective study of patients with active and inactive LS skin lesions. A standardized evaluation of a single designated study lesion per subject was performed at 3 visits. We evaluated the pattern and correlation between assessed features and physician's global assessments of activity (PGA-A).

Results: Ninety of 103 subjects had evaluable data; 66 had active and 24 inactive disease. Subjects had similar age of onset, sex, and disease patterns. Linear scleroderma was the most common subtype. Features specific for active disease included erythema, violaceous color, tactile warmth, abnormal skin texture, and disease extension. Scores for these variables changed over time and correlated with PGA-A of the lesion. Active and inactive lesions could not be distinguished by the presence or level of skin thickening, either of lesion edge or center. However, in active lesions, skin thickening scores did correlate with PGA-A scores. Regression analysis identified the combination of erythema, disease extension, violaceous color, skin thickening, and abnormal texture as predictive of PGA-A at study entry. Damage features were common irrespective of activity status.

Conclusion: We identified variables strongly associated with disease activity, expanding upon those used in current measures, and determined their relative importance in physician activity scoring. Skin thickening was found to lack specificity for disease activity. These results will help guide development of a sensitive, responsive activity tool to improve care of patients with LS.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3899/jrheum.171381DOI Listing
December 2018

Morphea: Current concepts.

Clin Dermatol 2018 Jul - Aug;36(4):475-486. Epub 2018 Apr 10.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas, USA. Electronic address:

Morphea is an inflammatory, sclerosing skin disorder that can involve the underlying soft tissues. Although the cause of morphea remains poorly investigated, genetic predisposition, immune dysregulation, and environmental factors have been implicated. Morphea is associated with cosmetic and functional sequelae, and internal organ involvement is rare. Early diagnosis and treatment are imperative to minimize damage such as limitation of range of motion. This review summarizes advances in diagnosis and treatment of morphea, allowing clinicians to better serve patients with this condition.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clindermatol.2018.04.005DOI Listing
December 2018

Differentiating trigeminal motor neuropathy and progressive hemifacial atrophy.

Cutis 2018 01;101(1):E13-E14

Department of Dermatology, University of Texas Southwestern Medical Center at Dallas, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
January 2018

Skin mapping for the classification of generalized morphea.

J Am Acad Dermatol 2018 02;78(2):351-357

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:

Background: Generalized morphea lacks cohesive clinical features, limiting its clinical and investigative utility.

Objective: We sought to use computerized lesion mapping to objectively subtype morphea.

Methods: We conducted a 2-part cross-sectional study. In part 1, we created a discovery cohort of patients with generalized morphea of whom lesion maps were created to characterize subsets. Clinical and demographic features were compared between proposed subsets to determine if they identified clinically relevant differences. In part 2, we created a validation cohort to determine if proposed criteria were applicable to different individuals.

Results: A total of 123 patients with generalized morphea were included. Mapping produced 2 distribution patterns that encompassed the majority in both cohorts: isomorphic (areas of skin friction) and symmetric (symmetrically distributed on trunk/extremities). In the discovery cohort, the isomorphic subset was older (55.6 ± 12.7 vs 42.2 ± 20.1 years, P < .001), all female (30/30 vs 38/43, P = .05), and more often had lichen sclerosus changes (12/43 vs 8/43, P = .02); involvement of the reticular dermis, subcutaneous fat, and/or fascia was more common in symmetric (10/43 vs 1/30) (P = .02). These features persisted in the validation cohort.

Limitations: Single cohort was a limitation.

Conclusions: Symmetric and isomorphic subsets possess distinctive demographic and clinical features, suggesting they more accurately define the phenotype of generalized morphea. Consideration should be given to revising classification.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2016.08.052DOI Listing
February 2018

Skin care and cosmeceuticals: Attitudes and trends among trainees and educators.

J Cosmet Dermatol 2018 Apr 19;17(2):220-226. Epub 2017 Nov 19.

Department of Dermatology, UT Southwestern Medical Center, Dallas, TX, USA.

Introduction: Patients often seek skin care recommendations from their dermatologist. The objective of this study was to determine the degree of education dermatology residents receive on skin care and cosmeceutical products, the source of education, and the attitude of trainees and their educators toward skin care and cosmeceuticals.

Methods: A cross-sectional survey of dermatology residents and faculty via an online survey administered June 2015 and August 2015, respectively.

Results: In total, 104 dermatology residents and 50 dermatology faculty members completed the survey. Among the dermatology residents and faculty, equal distribution was represented across the country. The majority of residents and faculty (62% and 69%, respectively) report discussing skin care with up to 25% of their patients. Among resident participants, 76.5% "agree or strongly agree" that skin care and cosmeceutical education should be part of their education and the majority of residents (74.5%) report their education has been "too little or nonexistent" during residency. In contrast, the majority of the faculty (60%) reports their resident education is "just the right amount or too much" (P < .001).

Conclusions: Skin care and cosmeceutical recommendations are often discussed in dermatology visits. Dermatology residents feel that education on these products should be a part of their residency training.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/jocd.12460DOI Listing
April 2018

Transcriptional and Cytokine Profiles Identify CXCL9 as a Biomarker of Disease Activity in Morphea.

J Invest Dermatol 2017 08 24;137(8):1663-1670. Epub 2017 Apr 24.

University of Texas Southwestern Medical Center, Department of Dermatology, Dallas, Texas, USA. Electronic address:

IFN-related pathways have not been studied in morphea, and biomarkers are needed. We sought to characterize morphea serum cytokine imbalance and IFN-related gene expression in blood and skin to address this gap by performing a case-control study of 87 participants with morphea and 26 healthy control subjects. We used multiplexed immunoassays to determine serum cytokine concentrations, performed transcriptional profiling of whole blood and lesional morphea skin, and used double-staining immunohistochemistry to determine the cutaneous cellular source of CXCL9. We found that CXCL9 was present at increased concentrations in morphea serum (P < 0.0001), as were other T helper type 1 cytokines. CXCL9 serum concentration correlated with the modified Localized Scleroderma Skin Severity Index (r = 0.44, P = 0.0001), a validated measure of disease activity. CXCL9 gene expression was also increased in inflammatory lesional morphea skin (fold change = 30.6, P = 0.006), and preliminary transcriptional profiling showed little evidence for IFN signature in whole blood. Double-staining immunohistochemistry showed CXCL9 co-localized with CD68 dermal macrophages. In summary, inflammatory morphea is characterized by T helper type 1 cytokine imbalance in serum, particularly CXCL9, which is associated with disease activity. CXCL9 expression in lesional macrophages implicates the skin as the source of circulating cytokines. CXCL9 is a promising biomarker of disease activity in morphea.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jid.2017.04.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6217816PMC
August 2017

Histopathological changes in morphea and their clinical correlates: Results from the Morphea in Adults and Children Cohort V.

J Am Acad Dermatol 2017 Jun 9;76(6):1124-1130. Epub 2017 Mar 9.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:

Background: Histopathological features in morphea (localized scleroderma) and their clinical correlates are poorly described.

Objective: We sought to systematically describe histologic changes of morphea in a large, well-annotated cohort and determine the association between histopathology and clinical features.

Methods: This was a cross-sectional study of 83 patients enrolled in the Morphea in Adults and Children cohort. The main outcome measure was the association of microanatomical location and degree of sclerosis and inflammation seen on histologic samples with patient-reported symptoms and physician-based measures of severity.

Results: Pattern of sclerosis was associated with morphea subtype, the presence of patient-reported symptoms, and functional limitation. A bottom-heavy pattern of sclerosis was associated with pain and tightness (P = .0039 and .001, respectively). These symptoms were not associated with a top-heavy pattern. Severe inflammation may be associated with pain and functional limitation (P = .073 for both).

Limitations: Small sample size limits ability to detect associations, particularly in subgroups.

Conclusions: Histopathological examination of morphea may assist in identifying patients who may require additional monitoring and treatment. Features such as patterns of sclerosis and severity of inflammation should be included in pathology reports to help aid in clinical management.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2016.12.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5438266PMC
June 2017

Phototherapy for sclerosing skin conditions.

Clin Dermatol 2016 Sep-Oct;34(5):614-22. Epub 2016 May 20.

Department of Dermatology, University of Texas at Southwestern Medical Center, Dallas, TX. Electronic address:

Phototherapy is an effective treatment strategy for a variety of sclerosing skin conditions. There are a number of phototherapeutic modalities used for the treatment of sclerosing skin conditions, including ultraviolet (UV)A1, broadband UVA, psoralen plus UVA, and narrowband UVB phototherapy. As controlled trials with validated outcome measures are lacking for these therapies, existing evidence is largely level II for morphea and is even more minimal for scleroderma and other sclerosing disorders (scleroderma, lichen sclerosus, and chronic graft-versus-host disease, among others). Studies do suggest that phototherapy may be effective for many of these disorders, including those that have been unresponsive to other therapies. Phototherapy remains an attractive therapeutic option for patients due to its efficacy and favorable risk-versus-benefit profile. Phototherapy also offers a therapeutic alternative to systemic immunosuppressives for patients who cannot tolerate these medications.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clindermatol.2016.05.012DOI Listing
May 2017

Myeloid-Derived Suppressor Cells in Psoriasis Are an Expanded Population Exhibiting Diverse T-Cell-Suppressor Mechanisms.

J Invest Dermatol 2016 09 25;136(9):1801-1810. Epub 2016 May 25.

Department of Dermatology, The University of Texas Southwestern Medical Center, Dallas, Texas, USA. Electronic address:

Psoriasis vulgaris is an inflammatory skin disease caused by hyperactivated T cells regulated by positive and negative mechanisms; although the former have been much studied, the latter have not. We studied the regulatory mechanism mediated by myeloid-derived suppressor cells (MDSCs) and showed that MDSCs expanded in melanoma patients express dendritic cell-associated heparan sulfate proteoglycan-dependent integrin ligand, a critical mediator of T-cell suppressor function. We examined expansion of DC-HIL(+) MDSCs in psoriasis and characterized their functional properties. Frequency of DC-HIL(+) monocytic MDSCs (CD14(+)HLA-DR(no/low)) in blood and skin was markedly increased in psoriatic patients versus healthy control subjects, but there was no statistically significant relationship with disease severity (based on Psoriasis Area and Severity Index score). Blood DC-HIL(+) MDSC levels in untreated patients were significantly higher than in treated patients. Compared with melanoma-derived MDSCs, psoriatic MDSCs exhibited significantly reduced suppressor function and were less dependent on DC-HIL, but they were capable of inhibiting proliferation and IFN-γ and IL-17 responses of autologous T cells. Psoriatic MDSCs were functionally diverse among patients in their ability to suppress allogeneic T cells and in the use of either IL-17/arginase I or IFN-γ/inducible nitric oxide synthase axis as suppressor mechanisms. Thus, DC-HIL(+) MDSCs are expanded in psoriasis patients, and their mechanistic heterogeneity and relative functional deficiency may contribute to the development of psoriasis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jid.2016.02.816DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4992618PMC
September 2016

Correspondence: The association between morphea profunda and monoclonal gammopathy: A case series.

Dermatol Online J 2016 Mar 16;22(3). Epub 2016 Mar 16.

University of Wisconsin.

It is known that eosinophilic fasciitis can be associated with monoclonal gammopathy. There is clinical similarity between eosinophilic fasciitis and morphea profunda, but it is unclear whether morphea profunda might be associated with monoclonal gammopathy. The temporal quantification of gammopathy in morphea profunda has not been well characterized. We describe four patients with morphea profunda that were associated with monoclonal gammopathy. Three were associated with monoclonal IgG protein and one with IgM. No patients in our series developed myeloma. In conclusion, the association of monoclonal gammopathy is not unique to eosinophilic fasciitis and scleromyxedema. Further studies are necessary to characterize further the relationship between the two conditions.
View Article and Find Full Text PDF

Download full-text PDF

Source
March 2016

Dopamine efflux in response to ultraviolet radiation in addicted sunbed users.

Psychiatry Res Neuroimaging 2016 May 7;251:7-14. Epub 2016 Apr 7.

Department of Psychiatry, University of Texas Southwestern, Dallas, TX, USA; VA North Texas Health Care System, Dallas, TX, USA. Electronic address:

Compulsive tanning despite awareness of ultraviolet radiation (UVR) carcinogenicity may represent an "addictive" behavior. Many addictive disorders are associated with alterations in dopamine (D2/D3) receptor binding and dopamine reactivity in the brain's reward pathway. To determine if compulsive tanners exhibited neurobiologic responses similar to other addictive disorders, this study assessed basal striatal D2/D3 binding and UVR-induced striatal dopamine efflux in ten addicted and ten infrequent tanners. In a double-blind crossover trial, UVR or sham UVR was administered in separate sessions during brain imaging with single photon emission computerized tomography (SPECT). Basal D2/D3 receptor density and UVR-induced dopamine efflux in the caudate were assessed using (123)I-iodobenzamide ((123)I-IBZM) binding potential non-displaceable (BPnd). Basal BPnd did not significantly differ between addicted and infrequent tanners. Whereas neither UVR nor sham UVR induced significant changes in bilateral caudate BPnd in either group, post-hoc analyses revealed left caudate BPnd significantly decreased (reflecting increased dopamine efflux) in the addicted tanners - but not the infrequent tanners - during the UVR session only. Bilateral ∆BPnd correlated with tanning severity only in the addicted tanners. These preliminary findings are consistent with a stronger neural rewarding response to UVR in addicted tanners, supporting a cutaneous-neural connection driving excessive sunbed use.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pscychresns.2016.04.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5241090PMC
May 2016

The significant health threat from tanning bed use as a self-treatment for psoriasis.

J Am Acad Dermatol 2016 May;74(5):1015-7

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address:

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaad.2015.09.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4834431PMC
May 2016