Frontal fibrosing alopecia: a case series of 65 patients seen in a single Italian centre

Abstract

Background Frontal fibrosing alopecia (FFA) is increasing in prevalence worldwide and several series from different countries have been published, in order to detect possible causes of the disease outbreak. Objective To analyze the epidemiological, clinical and trichoscopic features of FFA seen in an Italian Dermatology Unit. Methods Clinical, trichoscopy, histopathology and medical details of all patients were compared with literature and general population data. Results From 2005 to 2017, we diagnosed FFA in 65 Caucasian females, with the number of diagnoses per year progressively rising. Premenopausal onset was detected in 21%. Frontal hair line recession was associated with parietal involvement in 80% of cases, occipital in 12.3% and eyebrows alopecia in 86.1%. In six cases, eyebrow alopecia preceded hair loss. Non‐inflammatory facial papules were detected in 1/3 of the patients. Itching was reported by 2/3 of the patients and was indicative of disease progression. Trichoscopy showed empty follicles/yellow dots, absence of follicular ostia, mild follicular hyperkeratosis, perifollicular erythema and ‘lonely hair’. Scalp lichen plano‐pilaris was seen in 15 patients, female pattern hair loss in 22. Therapy included short‐term treatment with systemic or intralesional corticosteroids followed by therapy with 5α‐reductase inhibitors of hydroxychloroquine and topical drugs. Arrest of FFA progression was seen in 75% of the patients, while 16 experienced worsening of the hairline despite therapy. Conclusions Frontal fibrosing alopecia not rarely starts before menopause, loss of the eyebrows can be the first sign of the disease, about 50% of the patients have other autoimmune conditions, FFA severity is not related to its duration, itching, follicular hyperkeratosis and erythema at trichoscopy are signs of disease progression, 25% of the patients show progression of hairline recession despite therapy.

Trichoscopy of primary cicatricial alopecias: an updated review

“…found a statistical association of perifollicular erythema with pruritus and proposed that it could represent a marker of activity of FFA. Similarly, another large series study by Starace et al . among 65 FFA patients found that the presence of scalp itching is associated with follicular hyperkeratosis and perifollicular erythema in trichoscopy indicating progressive disease which should lead to steroid therapy.…”

“…The severity of inflammation in FFA tends to be less intense than in LPP, which could be the reason for the thinner or milder perifollicular scales seen in FFA compared to LPP . Presence of perifollicular erythema as a marker of disease activity has been reported in multiple trichoscopic studies of FFA . This perifollicular erythema could disappear due to the pressure of the dermatoscope on the skin, hence gentle attachment is necessary .…”

“…45 In a large multicentre trichoscopic study of FFA involving 238 patients, Fernandez-Crehuet et al 37 found a statistical association of perifollicular erythema with pruritus and proposed that it could represent a marker of activity of FFA. Similarly, another large series study by Starace et al 44 among 65 FFA patients found that the presence of scalp itching is associated with follicular hyperkeratosis and perifollicular erythema in trichoscopy indicating progressive disease which should lead to steroid therapy. Fernandez-Crehuet et al 37 also found the statistic association between the cicatricial white patches and the severity of FFA.…”

“…3,5 Presence of perifollicular erythema as a marker of disease activity has been reported in multiple trichoscopic studies of FFA. 3,24,37,44 This perifollicular erythema could disappear due to the pressure of the dermatoscope on the skin, hence gentle attachment is necessary. 45 In a large multicentre trichoscopic study of FFA involving 238 patients, Fernandez-Crehuet et al 37 found a statistical association of perifollicular erythema with pruritus and proposed that it could represent a marker of activity of FFA.…”

Finasteride in Frontal Fibrosing Alopecia: Joining the Dots!

“…In recent years, the prevalence of FFA is being increasingly recognized in premenopausal women and men [6]. A study on serum sex hormone levels in 43 premenopausal women has reported no consistent alterations, suggesting that sex hormone levels are not directly implicated in the pathogenesis of FFA [7].…”