• Skip to main content
  • Skip to footer

PeDRA

Pediatric Dermatology Research Alliance

  • DONATE
  • Pay 2023 Membership Dues
  • Join PeDRA
  • Login
MENUMENU
  • Contact Us
  • For Patients & Families
        • Patient Support
        • Patient Education
        • Community Spotlight
        • Play Eczema Counts Now!
        • Patient Advisory Committee
        • Find a Pediatric Dermatologist (from SPD)
        • Parents, advocates, and patients over the age of 18 with a direct connection or significant interest in childhood skin disease or pediatric dermatology research are encouraged to join the PeDRA network for free as Community Members.

          Apply Now!

  • Education and Events
        • Virtual Education
        • PeDRA Podcasts
        • Points of Discussion Podcast
        • Mentorship Program
        • SPD/PeDRA Journal Club
        • 2022 PeDRA Annual Conference
        • Dash for Discovery Fun Run/Walk
        • Early Investigator Support
        • Play Eczema Counts Now!
        • Events Calendar
        • Save the Date! The 2023 PeDRA Annual Conference will be held November 9-11, 2023 at the Westin Buckhead Atlanta in Atlanta, GA.

           

          Listen to PeDRA’s newest podcast series, Industry 101!

  • Research
        • Grants and Fellowships
        • Resources for Researchers
        • Survey Application
        • Study Application
        • Research Areas
        • Publications
        • Now accepting applications for the Emerging Investigator Research Grants Program and Research Fellowships Program.

  • About
        • Mission & Vision
        • Leadership & Staff
        • Annual Reports & Financials
        • News Archive
        • Our Supporters
        • Our Membership
        • Our Committees
        • Getting to Know You
        • 2022 marked PeDRA’s 10th year of pursuing the vision of changing lives and healing children with skin disease through collaboration and discovery. Visit our 10th Anniversary Page to learn more about the origin of PeDRA and the many accomplishments during the first ten years!

  • Member Login

Connective Tissue & Autoimmune

Characterization of non-coding genetic loci and transcription factors that drive development of linear morphea

June 6, 2022 By Katherine Devenport

Fellow

Jennifer Sui
UC San Diego School of Medicine

Mentor

Dawn Eichenfield, MD, PhD
UC San Diego School of Medicine

Overview

Linear morphea is a skin disease that causes hardened, discolored bands on the skin. Though this disease is relatively uncommon, it usually affects children and can lead to significant deformities and disabilities when the child’s face, scalp, or joints are affected. The cause of linear morphea is not yet known. It is thought to be an immune disease that is affected by many factors, including the information contained in our genes. The purpose of this study is to help us understand what exactly in certain people’s genes causes them to develop linear morphea. We will use scientific tools to compare the DNA, and molecules that interact with DNA, in affected versus normal skin of children with linear morphea. We will also determine what effects these differences have on skin cells. By doing this, we will be able to better understand the disease and create more effective ways to treat it.

Status

This project was funded through a 2022 PeDRA Research Fellowship Grant.

Filed Under: Connective Tissue & Autoimmune

Characterization of the presenting cutaneous manifestations of juvenile dermatomyositis

April 6, 2022 By Katherine Devenport


Principal Investigator:

Sonia Kamath, MD
Children’s Hospital Los Angeles
Keck School of Medicine of the University of Southern California (USC)

Overview:

Juvenile dermatomyositis (JDM) is a rare autoimmune disease, causing muscle weakness and rash. The skin findings are variable and are crucial to establishing the diagnosis, as they often occur months or years prior to muscle weakness. Some children have a classic rash affecting the eyelids and knuckles, while others have less common findings, such as scalp rash. To better understand the skin disease in JDM, we propose to evaluate the spectrum of initial skin findings. We will conduct a multi-center, retrospective review of children diagnosed with JDM to determine the prevalence of skin findings at initial presentation, whether the presenting skin findings can help identify patients who develop muscle disease, and whether skin findings vary by race/ethnicity. Our findings will inform future prospective studies to define distinct presentations of JDM, characterize long-term disease outcomes, and improve treatment approaches for JDM.

Status:

This project was funded by a 2020 PeDRA Research Grant.

Filed Under: Connective Tissue & Autoimmune

Identifying Dysregulated Immune-Related Gene Networks at the Single-Cell Level in Pediatric Morphea

May 18, 2021 By Katherine Devenport

Fellow

Henry Chen
UT Southwestern Medical Center

Mentor

Heidi Jacobe MD, MSCS
UT Southwestern Medical Center

Overview

Morphea, or localized scleroderma, is an idiopathic disorder of the skin and underlying tissue that produces permanent disability and functional impairment due to joint contracture and soft tissue loss. Notably, pediatric-onset morphea is associated with more severe features compared to adult-onset morphea, such as worsened morbidity and prolonged duration of disease, impairing growth and resulting in disfigurement. Morphea has remitting and relapsing periods of activity, marked by inflammation and fibrosis, and damage which produces atrophy. Activity is characterized by an inflammatory dermal and subcutaneous lymphocytic infiltrate manifesting clinically as erythema and edema. Inflammatory activity often overlaps with a fibrotic phase distinguished by dense collagen deposition with infiltration of inflammatory cells manifesting clinically as hardened, discolored plaques. Fibrosis and resultant atrophy of the skin, soft tissue, and bone result in functional impairment. As with other autoimmune disorders, the predominant driver of long-term morphea damage and disability is thought to be the magnitude and duration of the initial active phase, which likely facilitates subsequent damaging fibrosis. However, the dysregulation of immune and fibrotic pathways at single-cell resolution that contribute to these changes has not been systematically studied. The only widely tested treatment for morphea is a combination of corticosteroids and methotrexate, the use of which is substantially limited by toxicity, particularly in the pediatric population. Therefore, characterization of immune pathway aberrations at the single-cell level is quintessential in the identification of molecular targets for therapeutic development.

Aim 1: Identify dysregulated immune networks in morphea relative to matched healthy control skin.

Aim 2: Utilize identified dysregulated immune networks to determine association with disease trajectory and therapeutic response.

Status

This project was funded through a 2021 PeDRA Research Fellowship Grant.

Filed Under: Connective Tissue & Autoimmune

Optimizing Clinical Application of Lymphocyte Activation Tests for the Detection of Causative Medications in severe cutaneous Adverse Reactions

April 13, 2021 By Katherine Devenport


Principal Investigator:

Reid Oldenburg, MD, PhD
University of California, San Diego

Overview:

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are catastrophic drug reactions that destroy the skin, surface of the eyes and lining of the mouth and genitals. Antibiotics, ibuprofen and anti-seizures medications can cause SJS and TEN. When these severe cutaneous adverse reactions occur, the causative medication must be stopped immediately. However, identifying a causative medication can be difficult, especially when children are taking multiple medications.

There is no available blood test that can identify drugs that cause SJS and TEN, making it difficult for doctors to continue prescribing important medications, or use similar medications. Our long term interest is to develop diagnostic blood tests that can safely be used to identify causative medications for SJS and TEN patients. Our current project will stimulate blood from SJS and TEN patients with known causative medications to identify inflammatory molecules that can be later be used to develop diagnostic tests.

Status:

This project was funded by a 2020 PeDRA Research Grant.

Filed Under: Connective Tissue & Autoimmune

Characterization of the presenting cutaneous manifestations of juvenile dermatomyositis

April 13, 2021 By Katherine Devenport


Principal Investigator:

Sonia Kamath, MD
Children’s Hospital Los Angeles
Keck School of Medicine of the University of Southern California (USC)

Overview:

Juvenile dermatomyositis (JDM) is a rare autoimmune disease, causing muscle weakness and rash. The skin findings are variable and are crucial to establishing the diagnosis, as they often occur months or years prior to muscle weakness. Some children have a classic rash affecting the eyelids and knuckles, while others have less common findings, such as scalp rash. To better understand the skin disease in JDM, we propose to evaluate the spectrum of initial skin findings. We will conduct a multi-center, retrospective review of children diagnosed with JDM to determine the prevalence of skin findings at initial presentation, whether the presenting skin findings can help identify patients who develop muscle disease, and whether skin findings vary by race/ethnicity. Our findings will inform future prospective studies to define distinct presentations of JDM, characterize long-term disease outcomes, and improve treatment approaches for JDM.

Status:

This project was funded by a 2020 PeDRA Research Grant.

Filed Under: Connective Tissue & Autoimmune

Investigating Constitutive and Cell-Driven Fibroblast Activation in Pediatric Morphea

May 11, 2020 By Katherine Devenport

Fellow

Laila Abbas
UT Southwestern Medical Center

Mentor

Heidi Jacobe, MD, MSCS
UT Southwestern Medical Center

Overview

Morphea, or localized scleroderma, is a neglected autoimmune disease of the skin and underlying tissue that produces permanent disability due to joint contracture and soft tissue loss. Approximately half of patients have disease onset in childhood, and pediatric morphea is associated with significant morbidity, with reported complications including loss of range of motion, joint deformity, facial disfigurement, and neurologic manifestations. Morphea has remitting and relapsing activity, marked by inflammation and fibrosis, and damage which produces atrophy. The overarching hypothesis of this project is that fibroblasts are constitutively activated in morphea and that both T cells and macrophages will promote activation of fibroblasts.

Aim 1: Determine constitutive expression of pro-fibrotic and inflammatory markers in fibroblasts derived from morphea lesions as compared to matched control specimens.

Aim 2: Co-cultures will be performed with combinations of healthy and morphea fibroblasts along with healthy and morphea derived T cells and macrophages to determine if these cells can potentiate fibroblast activation.

Status

This project was funded through a 2020 PeDRA Research Fellowship Grant.

Filed Under: Connective Tissue & Autoimmune

Genetic Variations in Non-coding Regulatory Regions in Linear Morphea

November 10, 2019 By Mike Siegel

Principal Investigator:

Dawn Eichenfield, MD, PhD
University of California, San Diego

Overview:

Morphea is an inflammatory skin condition leading to hardened, discolored, and ultimately, scarred skin. It has several different presentations. Linear morphea commonly appears in children as a depressed or bound-down line on the forehead or extremity. Exactly why morphea occurs is unknown; however, studies suggest it is caused by a combination of genetic and environmental factors. Our research aims to uncover the genetic causes of linear morphea. Where our study differs from prior studies is the focus on studying parts of the genome that do not code for proteins, but instead are involved in regulating when or how genes are active. It is only recently that we realized the importance and disease relevance of these “non-coding” portions and could feasibly study their effects. Our project will utilize the latest sequencing technologies to investigate genetic mutations in morphea that can lead to improved diagnosis and earlier treatment.

Status:

This study was funded as a 2018 PeDRA Pilot Grant and is currently underway.

Filed Under: Active Studies, Connective Tissue & Autoimmune

Sun Protection for Pediatric Rheumatology Patients

November 10, 2019 By Mike Siegel

Principal Investigator:

Irene Lara-Corrales, MD
University of Toronto

Overview:

This is a survey study in collaboration with the Childhood Arthritis and Rheumatology Research Alliance (CARRA). Pediatric rheumatologists and pediatric rheumatology patients will be surveyed about the sun protection knowledge to identify practice gaps and target areas for education.

Status:

Survey instrument was deployed in March 2021.

Filed Under: Active Studies, Connective Tissue & Autoimmune

  • Go to page 1
  • Go to page 2
  • Go to Next Page »

Footer

  • Our Membership
  • For Patients
  • For Researchers
  • Governance Policies
  • Financial Conflict of Interest Policy
  • Jobs at PeDRA
  • Facebook
  • Instagram
  • LinkedIn
  • Twitter
  • YouTube
Sign up to receive emails from PeDRA
Join PeDRA
Donate

Copyright © 2023 · Pediatric Dermatology Research Alliance (PeDRA) · All Rights Reserved. · Privacy Policy