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

Salido‐Vallejo, Rafael; Ruano, Juan; Garnacho‐Saucedo, Gloria; Godoy-Gijón, E.; Llorca, D.; Gómez‐Fernández, Cristina; Moreno‐Giménez, J.C.

doi:10.1111/ced.12398

Cited by 26 publications

(27 citation statements)

References 18 publications

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“…In this study, we utilized the FASI, which was newly designed to measure specifically the clinical severity of TSC-associated angiofibromas [12]. It allowed a more reliable scoring for severities and improvements of facial angiofibromas after the treatment.…”

Section: Discussionmentioning

confidence: 99%

“…In 2014, Salido-Vallejo et al [12] published a study on developing a reliable assessment tool to measure the severity and responsiveness to therapy for TSC-associated facial angiofibroma. The Facial Angiofibroma Severity Index (FASI) is obtained by summing the partial scores assigned to each of 3 features that are characteristic for the evaluation of facial angiofibroma: erythema, size, and extent of the lesion.…”

Section: Introductionmentioning

confidence: 99%

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Comparative Effects of Topical 0.2% Sirolimus for Angiofibromas in Adults and Pediatric Patients with Tuberous Sclerosis Complex

et al. 2018

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Background: Recent reports have suggested that the topical formulation of sirolimus is effective in treating facial angiofibromas in tuberous sclerosis complex (TSC). Here, we determined the safety and efficacy of 0.2% topical sirolimus for the treatment of facial angiofibroma and compared its effects based on age. Method: This was a retrospective study which involved 36 TSC patients with facial angiofibromas who were treated with 0.2% sirolimus ointment. Its effect was evaluated using the Facial Angiofibroma Severity Index (FASI). In order to observe its comparative effect based on patient age, a subgroup analysis was performed, between the adult group (> 18 years old) and the pediatric group (≤18 years old). Results: The total FASI as well as its subcategories (erythema, size, and extent) showed statistically significant improvements after the topical treatment with 0.2% sirolimus ointment (FASI before treatment: 7.2 ± 1.1, FASI after treatment: 4.4± 1.4, p < 0.001). Among the subcategories of FASI, the erythema was most significantly reduced with the fastest response to the treatment. In a subgroup analysis, the pediatric group showed significantly greater improvements in FASI (improvement of FASI in the pediatric group = 49.7 ± 12.2%, adult group = 27.9 ± 15.6%, p < 0.001). The serial improvement analysis also showed that the pediatric group achieved a consistently greater improvement in FASI at all visits. Its 1-year application in 3 patients demonstrated a continuous maintenance effect. No significant adverse effects were observed. Conclusion: 0.2% sirolimus ointment is safe and effective for facial angiofibromas. Considering its higher efficacy in younger patients, an early initiation of the treatment is recommended.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Comparative Effects of Topical 0.2% Sirolimus for Angiofibromas in Adults and Pediatric Patients with Tuberous Sclerosis Complex

et al. 2018

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“…That scale has just recently been validated and published. [21] So we graded the skin lesions in papules, nodules and erythema and discussed the photos with each other in order to reach consensus about our grading system. We used an oral rapamycin solution, which might have given more side effects.…”

Section: Discussionmentioning

confidence: 99%

Topical rapamycin for angiofibromas in patients with tuberous sclerosis: how does it work in clinical practice?

Ekbäck¹,

Edström²

2016

PAR

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How to cite this article: Palmetun Ekbäck M, Wiegleb Edström D. Topical rapamycin for angiofibromas in patients with tuberous sclerosis: how does it work in clinical practice? Plast Aesthet Res 2016;3:328-34.Aim: Topical rapamycin for angiofibromas has been reported to be a new promising treatment. This study aims to report the outcome in clinical practice. Methods: A retrospective clinical follow-up on twenty-three patients who had been prescribed an oral solution of 0.1% rapamycin, to be applied on facial lesions once a day. Results: Seventeen of 23 patients continued the treatment. Papules and nodules were improved in 8 patients (47%) and erythema in 12 (70%). Side effects, such as stinging and redness were reported in 35% of patients. Blood samples were taken from 5 patients and no rapamycin could be detected. All patients who paused the treatment relapsed. Conclusion: Topical rapamycin has a positive effect on angiofibromas with improvement in both erythema and papules even if only applied every second to third day, but continuous treatment is needed. ABSTRACTArticle history:

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“…It was based on subjective improvement as noted by patients and family in minors, whereas objective improvement was noted as a decrease in erythema and size of papules in sequential photographs. Efficacy was graded according to FA severity index (FASI),[ 1 ] which is obtained by adding partial scores for erythema, size of lesions, and extent of lesions of FA [ Table 1 ].…”

Section: Methodsmentioning

confidence: 99%

Use of topical rapamycin in facial angiofibromas in Indian skin type

2016

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Introduction:Facial angiofibromas (FA) are the most visible cutaneous manifestations in patients with tuberous sclerosis (TS), often resulting in stigmatization of the affected individuals. Recent studies have suggested that topical rapamycin may be an effective treatment for angiofibromas.Aim:To study the safety and efficacy of topical rapamycin in treatment of FA in Type IV–VI skin type.Materials and Methods:Five female patients with FA were included in the study, four of whom had TS, whereas one had isolated angiofibromas without systemic involvement. The age of the patients varied from 6 to 44 years. After baseline evaluation, they were advised to apply topical rapamycin (0.1–1%) in white soft paraffin base twice daily. Follow-up varied from 1 month to 6 months and is ongoing.Results:A sustained improvement was observed with respect to erythema, size as well as extent of the lesions as early as within 2 weeks of starting treatment. No side effects were observed. A correlation between duration of angiofibromas and effectiveness of treatment was noted.Conclusion:Topical rapamycin appears to be a safe and effective alternative to surgical or laser-based treatments in patients with FA. This treatment shows potential to be a first-line management for FA and appears safe to start in early childhood.

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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

Abstract: 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.

Cited by 26 publications

References 18 publications

Comparative Effects of Topical 0.2% Sirolimus for Angiofibromas in Adults and Pediatric Patients with Tuberous Sclerosis Complex

Comparative Effects of Topical 0.2% Sirolimus for Angiofibromas in Adults and Pediatric Patients with Tuberous Sclerosis Complex

Topical rapamycin for angiofibromas in patients with tuberous sclerosis: how does it work in clinical practice?

Use of topical rapamycin in facial angiofibromas in Indian skin type

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