Development and validation of the Amyloidosis Area and Severity Index for primary cutaneous amyloidosis
- 1Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China
- 2Correspondence to: Wenyan He, MD, PhD, Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, No.1, Youyi Rd, Yuanjiagang, Yuzhong District, Chongqing, 400016, China.
Abstract
Background: Primary cutaneous amyloidosis (PCA) is a chronic, pruritic skin disorder lacking a standardized severity instrument, hindering clinical assessment and research. Objectives: To develop and validate a novel clinician-reported outcome measure, the Amyloidosis Area and Severity Index (AASI), for PCA. Methods: The AASI was developed via a modified Delphi consensus process informed by surveys of 340 patients and 33 dermatologists. In a prospective validation study involving 50 patients, 10 raters independently assessed PCA patients twice using the AASI. Interrater and intrarater reliability were determined using intraclass correlation coefficients, and construct validity by correlating AASI scores with established outcome measures. Results: The Delphi process prioritized 4 core domains: papule/macule, dryness/scaling, excoriation, and lichenification. The AASI demonstrated moderate interrater (intraclass correlation coefficient = 0.701) and good-to-excellent intrarater reliability (intraclass correlation coefficient range: 0.762-0.942). Construct validity was supported by strong correlations with Investigator's Global Assessment (ρ = 0.84) and Xerosis Score (ρ = 0.75) (all P < .001). Limitations: Delphi consensus restricted to Chinese clinicians and patients; single-center validation; treatment responsiveness being evaluated in an ongoing trial. Conclusions: The AASI is a new, consensus-derived severity instrument for PCA with moderate-to-good reliability and validity, providing a foundation for future standardized assessment for clinical and trial use.
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BibTeX
@article{ziqijiang2026development,
title = {{Development and validation of the Amyloidosis Area and Severity Index for primary cutaneous amyloidosis}},
author = {Ziqi Jiang, MD, PhD and Siru Wang, MS and Jianxia Xiong, MS and Shilin Chen, MS and Chengzhi Li, MS and Zelong Li, MS and Yun Pan, MS and Chuan Liu, MS and Rentao Yu, MD, PhD and Qian Liu, MD, PhD and Yujing Huang, MD, PhD and Songyao Deng, MS and Fengzeng Li, MD, PhD and Aijun Chen, MD, PhD and Wenyan He, MD, PhD},
journal = {JAAD International},
year = {2026},
month = oct,
doi = {10.1016/j.jdin.2026.09.009},
url = {https://doi.org/10.1016/j.jdin.2026.09.009},
note = {CC BY},
}
RIS
TY - JOUR
TI - Development and validation of the Amyloidosis Area and Severity Index for primary cutaneous amyloidosis
AU - Ziqi Jiang, MD, PhD
AU - Siru Wang, MS
AU - Jianxia Xiong, MS
AU - Shilin Chen, MS
AU - Chengzhi Li, MS
AU - Zelong Li, MS
AU - Yun Pan, MS
AU - Chuan Liu, MS
AU - Rentao Yu, MD, PhD
AU - Qian Liu, MD, PhD
AU - Yujing Huang, MD, PhD
AU - Songyao Deng, MS
AU - Fengzeng Li, MD, PhD
AU - Aijun Chen, MD, PhD
AU - Wenyan He, MD, PhD
PY - 2026
DA - 2026/10/01
JO - JAAD International
DO - 10.1016/j.jdin.2026.09.009
UR - https://doi.org/10.1016/j.jdin.2026.09.009
AB - Background: Primary cutaneous amyloidosis (PCA) is a chronic, pruritic skin disorder lacking a standardized severity instrument, hindering clinical assessment and research. Objectives: To develop and validate a novel clinician-reported outcome measure, the Amyloidosis Area and Severity Index (AASI), for PCA. Methods: The AASI was developed via a modified Delphi consensus process informed by surveys of 340 patients and 33 dermatologists. In a prospective validation study involving 50 patients, 10 raters independently assessed PCA patients twice using the AASI. Interrater and intrarater reliability were determined using intraclass correlation coefficients, and construct validity by correlating AASI scores with established outcome measures. Results: The Delphi process prioritized 4 core domains: papule/macule, dryness/scaling, excoriation, and lichenification. The AASI demonstrated moderate interrater (intraclass correlation coefficient = 0.701) and good-to-excellent intrarater reliability (intraclass correlation coefficient range: 0.762-0.942). Construct validity was supported by strong correlations with Investigator's Global Assessment (ρ = 0.84) and Xerosis Score (ρ = 0.75) (all P < .001). Limitations: Delphi consensus restricted to Chinese clinicians and patients; single-center validation; treatment responsiveness being evaluated in an ongoing trial. Conclusions: The AASI is a new, consensus-derived severity instrument for PCA with moderate-to-good reliability and validity, providing a foundation for future standardized assessment for clinical and trial use.
SN - 2666-3287
ER -