Development and validation of the Amyloidosis Area and Severity Index for primary cutaneous amyloidosis
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Published on: March 1, 2024
Ziqi Jiang1, Siru Wang1, Jianxia Xiong1
1Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.
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.
