Can you provide an overview of frontal fibrosing alopecia, including its epidemiology, clinical presentation, diagnostic work‑up, and first‑line treatment options?

Frontal Fibrosing Alopecia: Overview

What is Frontal Fibrosing Alopecia?

Frontal fibrosing alopecia (FFA) is a primary scarring (cicatricial) alopecia that causes progressive, irreversible recession of the frontotemporal hairline, predominantly affecting postmenopausal Caucasian women, and is considered a variant of lichen planopilaris. 1, 2

Epidemiology

• FFA was first described in 1994 and was once rare, but has increased dramatically to become the most common cause of cicatricial alopecia worldwide 2
• The condition predominantly affects postmenopausal Caucasian women, though it has been observed in premenopausal women and occasionally men 1
• Familial cases are reported with identified gene associations, though the exact pathophysiology remains controversial 2
• The increasing incidence is attributed to both improved recognition/awareness and potential environmental factors 1

Etiology

• The etiology remains elusive, with potential contributing factors including genetic predisposition, hormonal influences, and chemical exposures 1
• FFA is characterized by loss of follicular stem cells and fibrosis, distinguishing it from non-scarring alopecias 3
• The histology is identical to lichen planopilaris, though only a small number of patients have coincidental LPP, and the vast majority have no evidence of lichen planus elsewhere 2

Clinical Presentation

Primary Features

• The hallmark presentation is symmetrical, progressive recession of the frontotemporal hairline appearing as a scarring hairless band 1, 2
• Bilateral eyebrow loss frequently accompanies the hairline recession 1, 2
• Body hair involvement occurs less commonly 3
• The disease exhibits slow and distinctive progression over months to years 1, 4

Physical Examination Findings

• Look for an asymptomatic atrophic alopecia restricted to the frontal hairline with a band-like pattern 4
• Perifollicular erythema may be present in the early inflammatory phase (frontal effluvium) 4
• The symmetry and patterned nature of hair loss distinguish FFA from typical lichen planopilaris 2

Dermoscopy (Trichoscopy) Findings

• Dermoscopy aids in diagnosis by revealing characteristic features specific to FFA 1
• The examination should focus on identifying perifollicular changes and scarring patterns that differentiate FFA from other forms of alopecia 1

Diagnostic Work-Up

Clinical Diagnosis

FFA diagnosis is primarily clinical based on the characteristic pattern of frontotemporal hairline recession with or without eyebrow loss in the appropriate demographic 3, 1

Confirmatory Testing

• Scalp biopsy is the gold standard for confirmation, showing perifollicular lymphocytic infiltrate with fibrosis 4
• Biopsy should be performed when the diagnosis is uncertain or to confirm before initiating aggressive systemic therapy 4
• Histology reveals findings identical to lichen planopilaris, with loss of follicular stem cells and scarring 3, 2

Laboratory Testing

• No specific laboratory tests are diagnostic for FFA 2
• Consider thyroid function tests (TSH), ferritin, vitamin D, and zinc levels to exclude nutritional or hormonal contributors to hair loss, particularly if presentation is atypical 5
• Hormonal evaluation (testosterone, SHBG) may be warranted if signs of androgen excess are present 5

First-Line Treatment Options

Treatment Goals and Expectations

The primary goal of FFA treatment is disease stabilization and halting progression rather than hair regrowth, due to the irreversible scarring nature of the condition 3, 6

Highest Evidence Treatments

The three treatments with the highest level of evidence are 5-α-reductase inhibitors, intralesional corticosteroids, and hydroxychloroquine 3

5-α-Reductase Inhibitors (Finasteride/Dutasteride)

• These antiandrogen medications represent first-line systemic therapy for FFA 3
• They work by blocking conversion of testosterone to dihydrotestosterone, potentially slowing disease progression 3
• Finasteride is typically dosed at 2.5-5 mg daily in postmenopausal women 3

Intralesional Corticosteroids

• Intralesional steroids are first-line treatment, particularly effective in the early inflammatory phase 3, 4
• They should be administered when perifollicular erythema is present, indicating active inflammation 4
• Treatment should be initiated early to arrest disease in its inflammatory phase before irreversible scarring occurs 4

Hydroxychloroquine

• This antimalarial agent has high-level evidence for treating FFA 3
• It provides immunomodulatory effects that may slow disease progression 3
• Typical dosing is 200-400 mg daily with appropriate ophthalmologic monitoring 3

Adjunctive Topical Therapies

• Topical corticosteroids should be tried in early-stage FFA to arrest the inflammatory phase 4
• Topical corticosteroids, sometimes combined with minoxidil, can arrest hair loss when administered for at least 3 months 4
• Topical calcineurin inhibitors (tacrolimus, pimecrolimus) represent alternative immunomodulators for early disease 4

Other Treatment Options with Variable Evidence

• Systemic retinoids, pioglitazone, oral antibiotics (tetracyclines), excimer laser, and hair transplantation have been used but require further data to draw definitive conclusions 3
• Oral corticosteroids have conflicting results and significant side effect profiles 6

Critical Management Considerations

Common Pitfalls

• Without treatment, FFA is slowly progressive, though in most cases the disease may resolve spontaneously after several years 2, 4
• The response to treatment is often disappointing, with no single therapeutic regimen proven effective in stopping hairline recession and inducing hair growth 6
• No randomized controlled trials have been conducted for FFA treatment, so current recommendations are based on small retrospective studies 3, 6
• Hair transplantation should be approached cautiously, as the scarring nature and potential ongoing inflammation may compromise graft survival 3

Treatment Algorithm

• Initiate treatment early when perifollicular erythema is present, indicating active inflammation that is potentially reversible 4
• Start with combination therapy: topical corticosteroids plus either 5-α-reductase inhibitor or hydroxychloroquine for systemic control 3, 4
• Add intralesional corticosteroids to areas of active inflammation with erythema 3, 4
• Continue treatment for at least 3-6 months before assessing response, as hair follicle cycles require this timeframe 4
• If no stabilization occurs after 6 months, consider switching or adding alternative agents (hydroxychloroquine if on finasteride, or vice versa) 3

Monitoring and Follow-Up

• Assess disease progression by measuring hairline position and documenting with photography at each visit 1
• Monitor for treatment side effects, particularly with systemic agents (liver function with hydroxychloroquine, sexual side effects with finasteride) 3
• Set realistic expectations with patients that treatment aims to halt progression, not restore lost hair 3, 6