Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Mar-Apr;41(2):247-252.
doi: 10.1111/pde.15508. Epub 2024 Jan 17.

In-person validation of the Ichthyosis Scoring System

Caroline Echeandia-Francis  1 , Qisi Sun  2 , Sarah Asch  3 , Cheryl Bayart  4 , Latanya Benjamin  5 , Sarah D Cipriano  6 , Brittany Craiglow  1 , Jonathan Dyer  7 , Moise L Levy  8   9 , Evelyn Lilly  10 , Brandon Newell  11   12 , Jingchen Liang  13 , Geliang Gan  13 , Yanhong Deng  13 , Amy S Paller  14 , Keith A Choate  1   15   16
Affiliations

In-person validation of the Ichthyosis Scoring System

Caroline Echeandia-Francis et al. Pediatr Dermatol. 2024 Mar-Apr.

Abstract

Background: Ichthyoses 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.

Methods: Sixty-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.

Results: Intrarater 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.

Conclusions: ISS 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.

Keywords: body surface area; erythema; ichthyosis; pigmentation; telemedicine.

PubMed Disclaimer

References

REFERENCES

    1. Marukian NV, Deng Y, Gan G, et al. Establishing and validating an ichthyosis severity index. J Invest Dermatol. 2017;137(9):1834-1841. doi:10.1016/j.jid.2017.04.037
    1. Kamalpour L, Rice ZP, Pavlis M, Veledar E, Chen SC. Reliable methods to evaluate the clinical severity of ichthyosis. Pediatr Dermatol. 2010;27(2):148-153. doi:10.1111/j.1525-1470.2010.01114.x
    1. Paller AS, Renert-Yuval Y, Suprun M, et al. An IL-17-dominant immune profile is shared across the major orphan forms of ichthyosis. J Allergy Clin Immunol. 2017;139(1):152-165. doi:10.1016/j.jaci.2016.07.019
    1. Bodemer C, Bourrat E, Mazereeuw-Hautier J, et al. Short- and medium-term efficacy of specific hydrotherapy in inherited ichthyosis. Br J Dermatol. 2011;165(5):1087-1094. doi:10.1111/j.1365-2133.2011.10510.x
    1. Sun Q. ISS calculator [Mobile App]. Sujal Adhid/AppStore. 2020 Apps.apple.com/us/app/iss-calculator/id153108611

LinkOut - more resources