TY - JOUR
T1 - Development and Initial Validation of a Novel System to Assess Ichthyosis Severity
AU - Sun, Qisi
AU - Asch, Sarah
AU - Bayart, Cheryl
AU - Bayliss, Susan J.
AU - Benjamin, Latanya
AU - Bruckner, Anna
AU - Digiovanna, John J.
AU - Fleckman, Philip
AU - Funk, Tracy
AU - Lucky, Anne
AU - Nelson, Caroline A.
AU - Newell, Brandon
AU - Polcari, Ingrid
AU - Teng, Joyce
AU - Williams, Mary L.
AU - Gan, Geliang
AU - Deng, Yanhong
AU - Paller, Amy S.
AU - Choate, Keith A.
N1 - Publisher Copyright:
© 2022 American Medical Association. All rights reserved.
PY - 2022/4
Y1 - 2022/4
N2 - 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 ichthyosis severity across the entire body. Objective: To create and evaluate a comprehensive and user-friendly instrument to measure total body ichthyosis severity in adults and children. Design, Setting, Participants: In this qualitative study, ichthyosis experts participated in the content development of the Ichthyosis Scoring System (ISS). The body was divided into 10 regions, and Likert scales (0-4) were created to quantify scale and erythema, with extensive descriptors and photographic standards. An 83-image teaching set was created from photographs of participants with ichthyosis. Two cohorts of dermatologists (11 total) independently scored all test photographs twice to evaluate interrater and intrarater reliabilities. Participants were enrolled worldwide from referral centers and patient advocacy groups. Participants of all ages, races, and ethnicities were included in the creation of ISS, and dermatologists with varying experience and areas of expertise participated as raters to evaluate the ISS. The study was conducted from 2019 to 2021, and the data were analyzed in 2021. Main Outcomes and Measures: Intraclass correlation coefficients determined overall reliabilities. Results: Across both cohorts of 11 dermatologists in total, the intraclass correlation coefficients for total, scale and erythema scores were greater than 0.90 (95% CI, 0.77-0.97), greater than 0.91 (95% CI, 0.79-0.98), and greater than 0.88 (95% CI, 0.72-0.97), respectively. Most body sites exhibited moderate to good interrater reliabilities for scale and erythema. Intrarater reliabilities were good to excellent. Conclusions and Relevance: The results of this qualitative study demonstrate reproducibility and suggest that the ISS is a reliable system to measure global ichthyosis severity in adults and children.
AB - 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 ichthyosis severity across the entire body. Objective: To create and evaluate a comprehensive and user-friendly instrument to measure total body ichthyosis severity in adults and children. Design, Setting, Participants: In this qualitative study, ichthyosis experts participated in the content development of the Ichthyosis Scoring System (ISS). The body was divided into 10 regions, and Likert scales (0-4) were created to quantify scale and erythema, with extensive descriptors and photographic standards. An 83-image teaching set was created from photographs of participants with ichthyosis. Two cohorts of dermatologists (11 total) independently scored all test photographs twice to evaluate interrater and intrarater reliabilities. Participants were enrolled worldwide from referral centers and patient advocacy groups. Participants of all ages, races, and ethnicities were included in the creation of ISS, and dermatologists with varying experience and areas of expertise participated as raters to evaluate the ISS. The study was conducted from 2019 to 2021, and the data were analyzed in 2021. Main Outcomes and Measures: Intraclass correlation coefficients determined overall reliabilities. Results: Across both cohorts of 11 dermatologists in total, the intraclass correlation coefficients for total, scale and erythema scores were greater than 0.90 (95% CI, 0.77-0.97), greater than 0.91 (95% CI, 0.79-0.98), and greater than 0.88 (95% CI, 0.72-0.97), respectively. Most body sites exhibited moderate to good interrater reliabilities for scale and erythema. Intrarater reliabilities were good to excellent. Conclusions and Relevance: The results of this qualitative study demonstrate reproducibility and suggest that the ISS is a reliable system to measure global ichthyosis severity in adults and children.
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U2 - 10.1001/jamadermatol.2021.5917
DO - 10.1001/jamadermatol.2021.5917
M3 - Article
C2 - 35171201
AN - SCOPUS:85124935960
SN - 2168-6068
VL - 158
SP - 359
EP - 365
JO - JAMA Dermatology
JF - JAMA Dermatology
IS - 4
ER -