Development and Initial Validation of a Novel System to Assess Ichthyosis Severity

Sun, Qisi; Asch, Sarah; Bayart, Cheryl B.; Bayliss, Susan J.; Benjamin, Latanya; Bruckner, Anna L.; DiGiovanna, John J.; Fleckman, Philip; Funk, Tracy; Lucky, Anne W.; Nelson, Caroline A.; Newell, Brandon; Polcari, Ingrid C.; Teng, Joyce; Williams, Mary L.; Gan, Geliang; Deng, Yanhong; Paller, Amy S.; Choate, Keith

doi:10.1001/jamadermatol.2021.5917

Cited by 5 publications

(14 citation statements)

References 13 publications

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“…These scale and erythema photographic standards were previously used to evaluate ISS performance in evaluating photographs. 6 Raters referenced high quality photographic prints of standards with paired text descriptors as they scored participants during inperson validation. Sixty-five participants and nine raters were divided into three groups (rooms 1-3) with three raters in each room and 20, 24 and 21 participants in rooms 1, 2 and 3, respectively.…”

Section: Methodsmentioning

confidence: 99%

“…The ISS assesses 10 body sites for scale and erythema, and a free mobile application, ISS calculator, can be used to calculate total scores of ichthyosis burden accounting for differences in proportional body surface area between adults and children. 5 The ISS demonstrated overall excellent reliability (ISS-S ICC = 0.956, 95% CI, 0.925-0.974 and ISS-E ICC = 0.913, 95% CI, 0.855-0.949) when evaluated using photographs of affected patients, 6…”

Section: Introductionmentioning

confidence: 97%

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In‐person validation of the Ichthyosis Scoring System

Echeandia‐Francis,

Sun,

Asch

et al. 2024

Pediatric Dermatology

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BackgroundIchthyoses 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.MethodsSixty‐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.ResultsIntrarater 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.ConclusionsISS 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.

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Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

In‐person validation of the Ichthyosis Scoring System

Echeandia‐Francis,

Sun,

Asch

et al. 2024

Pediatric Dermatology

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“…The accurate assessment of disease burden and severity is a critical first step toward innovative clinical trials. In this issue of JAMA Dermatology , Sun et al report a novel system for measuring ichthyosis severity: the Ichthyosis Scoring System (ISS). The ISS is a comprehensive and useful measure of ichthyosis severity.…”

mentioning

confidence: 99%

“…The ISS is unique in incorporating severity at sites of particular psychosocial and functional importance, including the face, palms, and soles. Sun et al have demonstrated its reproducibility and reliability by evaluating interrater and intrarater reliabilities. In addition, they have developed a mobile application to improve the feasibility of using the ISS in clinical and research settings .…”

mentioning

confidence: 99%

“…Sun et al have demonstrated its reproducibility and reliability by evaluating interrater and intrarater reliabilities. In addition, they have developed a mobile application to improve the feasibility of using the ISS in clinical and research settings . With recent developments in the understanding of the pathogenesis and treatment of ichthyoses, the role of outcome measures, such as the ISS, is more important now than ever.…”

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confidence: 99%

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Ichthyosis Scoring System—A Powerful Tool in the Era of Immune Pathway–Targeted Therapies for Ichthyosis

Akiyama

2022

JAMA Dermatol

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ers. In-person visits could be replaced by telephone or virtual visits and mobile phlebotomists and coordinators who travel directly to the participants' homes. 12 At the institutional level, dismantling inequitable insurance barriers could help ensure the most prestigious institutions are accessible to those underinsured, noninsured, or with insurance common to racial and ethnic minority patients in each community. Institutions must also demonstrate active antiracism work and scrutinize the ethics and actions of their leadership and donors. This includes, but is not limited to, renaming hospital buildings after physician leaders who are underrepresented in medicine; reallocating donations to support diversity, inclusion, and equity initiatives; and repaying a portion of royalties earned from medications (ie, tretinoin) directly to patients and families historically affected by the unethical trials and experimentation that developed these treatments.At the federal level, government entities must ensure race and ethnicity reporting and consider ways to mandate diver-sity within current studies. As corroborated in the report by Chen and colleagues, 1 the NIH has made strides in remedying this by mandating that NIH-funded clinical trials include and report the proportion of racial and ethnic minority groups and women included in these trials. 3 The FDA released guidelines in 2020 calling to increase "racial and ethnic minority" recruitment, though notably do not define these terms. 13 In addition, similar mandates for studies not funded by the NIH do not yet exist.Data from dermatology clinical trials over the last 10 years have made it clear that it is not enough to simply say that trial diversity is important. It is time for dermatologists to take action to ensure the true value of diversity in clinical trials to help achieve more generalizable outcomes, to increase access to new medications and services, and to explicitly welcome underserved and underrepresented patients by institutions. These actions are part of the important process of truth and reconciliation with the past. These individual, institutional, and federal actions are required to achieve a more equitable future in dermatology, one that is better for all patients.

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