How is the Frontal Fibrosing Alopecia (FFA) severity score calculated and used to guide treatment in a post‑menopausal woman?

Frontal Fibrosing Alopecia Severity Score

The Frontal Fibrosing Alopecia Severity Score (FFASS) is a validated clinical tool that quantifies disease severity by measuring frontotemporal hairline recession distance, eyebrow loss extent, and presence of inflammatory activity markers, and should be used to monitor treatment response in postmenopausal women with FFA. 1

Components of the FFASS

The FFASS incorporates three primary domains to assess disease severity 1:

• Hairline recession measurement: Quantifies the distance of frontotemporal hairline recession from the original hairline position 1
• Eyebrow involvement: Assesses the extent of eyebrow hair loss, which frequently accompanies scalp involvement 1
• Inflammatory activity markers: Evaluates presence of perifollicular erythema, which directly correlates with progressive disease 2

Validation and Reliability

The FFASS demonstrates robust psychometric properties 1:

• Excellent interobserver reliability with an intraclass correlation coefficient of 0.97 (95% CI: 0.95-0.99) 1
• Good intraobserver reliability with an intraclass correlation coefficient of 0.86 (95% CI: 0.7-0.95) 1
• Significant correlation with Patient's Global Assessment, occipital involvement, and the Lichen Planopilaris Activity Index 1

Clinical Application for Treatment Guidance

Initial Assessment

When evaluating a postmenopausal woman with suspected FFA 3:

• Document baseline FFASS before initiating any therapy to establish a reference point for monitoring 1
• Look specifically for perifollicular erythema, as its presence indicates active disease progression and necessitates more aggressive treatment 2
• Assess for "lonely hair sign" (isolated terminal hairs in areas of alopecia), which is characteristic of FFA 2
• Examine for eyebrow loss, particularly lateral portions, and body hair involvement 4, 3

Treatment Monitoring Algorithm

Use the FFASS at regular intervals (every 3-6 months) to determine treatment efficacy 1:

• Decreasing or stable FFASS with resolution of perifollicular erythema indicates successful disease stabilization—continue current therapy 2
• Increasing FFASS or persistent perifollicular erythema indicates treatment failure—escalate to alternative immunosuppressive therapy 2
• Stable FFASS without inflammatory signs for >12 months may warrant consideration of cautious drug withdrawal with close monitoring 2

First-Line Treatment Options Based on Evidence

The highest level of evidence supports 5:

• 5-α-reductase inhibitors (finasteride or dutasteride) as primary systemic therapy 5
• Intralesional corticosteroids for localized areas of active inflammation 5
• Hydroxychloroquine 400 mg daily as systemic anti-inflammatory therapy 5

Second-Line Options for Treatment Failures

When first-line therapies fail to stabilize FFASS 2:

• Methotrexate 15-25 mg weekly can achieve disease stabilization, though response may take 7-16 months 2
• Combination therapy with topical minoxidil may be added to any systemic regimen, though evidence is limited 2

Critical Pitfalls to Avoid

Do not rely solely on patient-reported improvement, as FFA has a variable course with potential for spontaneous stabilization that can lead to overestimating treatment effects 2. The FFASS provides objective measurement to distinguish true treatment response from natural disease fluctuation 1.

Do not discontinue treatment based on subjective improvement alone—use serial FFASS measurements and specifically assess for absence of perifollicular erythema before considering treatment cessation 2.

Recognize that hair regrowth is not the treatment goal in this scarring alopecia; disease stabilization (stable or improving FFASS) is the realistic endpoint 4, 5.

Prognostic Considerations

The FFASS helps stratify patients by severity 1:

• Higher baseline FFASS scores indicate more extensive disease and may require more aggressive initial therapy 1
• Persistent perifollicular erythema despite treatment predicts ongoing progression regardless of FFASS stability 2
• Occipital involvement correlates with higher FFASS and suggests more severe disease phenotype 1