Facial Angiofibroma Severity Index (FASI): reliability assessment of a new tool developed to measure severity and responsiveness to therapy in tuberous sclerosis-associated facial angiofibroma
Keywords: 
Tuberous sclerosis complex (TSC)
Autosomal dominant neurocutaneous disorder
Multisystem hamartomatous tumours
Facial angiofibroma (FA).
Issue Date: 
2014
Publisher: 
Oxford academic
ISSN: 
1365-2230
Citation: 
Salido-Vallejo, R. (Rafael); Ruano, J. (J.); Garnacho-Saucedo, G.M. (Gloria Maria); et al. "Facial Angiofibroma Severity Index (FASI): reliability assessment of a new tool developed to measure severity and responsiveness to therapy in tuberous sclerosis-associated facial angiofibroma". Clinical and Experimental Dermatology. 39 (8), 2014, 888 - 893
Abstract
Background. Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by the development of multisystem hamartomatous tumours. Topical sirolimus has recently been suggested as a potential treatment for TSC-associated facial angiofibroma (FA). Aim. To validate a reproducible scale created for the assessment of clinical severity and treatment response in these patients. Methods. We developed a new tool, the Facial Angiofibroma Severity Index (FASI) to evaluate the grade of erythema and the size and extent of FAs. In total, 30 different photographs of patients with TSC were shown to 56 dermatologists at each evaluation. Three evaluations using the same photographs but in a different random order were performed 1 week apart. Test and retest reliability and interobserver reproducibility were determined. Results. There was good agreement between the investigators. Inter-rater reliability showed strong correlations (> 0.98; range 0.97–0.99) with inter-rater correlation coefficients (ICCs) for the FASI. The global estimated kappa coefficient for the degree of intra-rater agreement (test–retest) was 0.94 (range 0.91–0.97). Conclusions. The FASI is a valid and reliable tool for measuring the clinical severity of TSC-associated FAs, which can be applied in clinical practice to evaluate the response to treatment in these patients.

Files in This Item:
File
ced0888.pdf
Description
Size
837.18 kB
Format
Adobe PDF


Statistics and impact

Items in Dadun are protected by copyright, with all rights reserved, unless otherwise indicated.