Uncommon subtypes of frontal fibrosing alopecia: retrospective analysis of clinical characteristics and prognosis

rontal fibrosing alopecia (FFA) is a type of progressive lymhocytic scarring alopecia described by Kossard in 1994,1 hich has become epidemic in recent years.2 It mainly ffects the frontal and temporal areas of hair implantation. hree typical patterns have been described to date: linear or type I), type II or diffuse (zigzag), and type III or pseudo ringe type. Each pattern has a different course, and the est prognosis is seen in the pseudo fringe pattern.3 pattern and three with ophiasic pattern, 83.3% of phototypes V and VI), and two patients had both lesions (one with male pattern and one with ophiasic pattern), with no statistical difference (p = 0.284 and 0.240 respectively). Thirteen patients were considered stable; 14 had active disease, and two were lost to follow-up (excluded), with the ophiasic pattern being the least stable (p = 0.054). The patients’ mean time of follow-up was 42.8 months (6--96 months, SD = 26.6). When comparing disease progression, the ophiasic pattern showed the most aggressive progression (2.25 cm/year, SD = 1.22; p = 0.026) with no statistical difference between the male pattern (1.02 cm/year, SD = 1 .07) and the plaque pattern (0.67 cm/year, SD = 0.51; p = 0.581). Similar findings were observed regarding disease stability (p = 0.054 for greater progression of the ophiasic pattern). Regarding the loss of eyebrows, the plaque pattern did not include patients with this type of alopecia (p < 0.001), and there was no statistical difference between the male and ophiasic patterns (p = 0.761), with 11 (91.7%) and seven patients (87.5%) with madarosis, respectively. The main comorbidities observed were arterial hypertension, diabetes mellitus, dyslipidemia, and hypothyroidism, which were frequent, as expected, in this age group, with no statistical differences between the groups. The demographic data, ethnicity, disease duration, and mean age at disease onset in our study were in line with other rare reports of atypical patterns of FFA.4,6 However, the proportion of each atypical pattern varies widely between studies. Kanti et al., in a series of 490 patients with FFA, found 32% with the ophiasic pattern (called the ‘‘band-like’’ pattern).8 Rossi et al. described this pattern in 6.1% of their patients with FFA. These authors also found 12.2% of patients with the male pattern and 2.0% with the plaque pattern (called the ‘‘headdress’’ pattern).6 Recently, Goldman et al. described a case of fibrosing frontal alopecia with a symmetrical ’epsilon-shaped’ alopecia pattern, which mimicked traction alopecia with symmetrical alopecia in the temporal area.9 In the present series, the plaque pattern, which would be the localized form of the disease, seems to have the best prognosis and the slowest evolution. These patients did not h p t

pattern and three with ophiasic pattern, 83.3% of phototypes V and VI), and two patients had both lesions (one with male pattern and one with ophiasic pattern), with no statistical difference (p = 0.284 and 0.240 respectively).
Thirteen patients were considered stable; 14 had active disease, and two were lost to follow-up (excluded), with the ophiasic pattern being the least stable (p = 0.054).
When comparing disease progression, the ophiasic pattern showed the most aggressive progression (2.25 cm/year, SD = 1.22; p = 0.026) with no statistical difference between the male pattern (1.02 cm/year, SD = 1 .07) and the plaque pattern (0.67 cm/year, SD = 0.51; p = 0.581). Similar findings were observed regarding disease stability (p = 0.054 for greater progression of the ophiasic pattern).
Regarding the loss of eyebrows, the plaque pattern did not include patients with this type of alopecia (p < 0.001), and there was no statistical difference between the male and ophiasic patterns (p = 0.761), with 11 (91.7%) and seven patients (87.5%) with madarosis, respectively.
The main comorbidities observed were arterial hypertension, diabetes mellitus, dyslipidemia, and hypothyroidism, which were frequent, as expected, in this age group, with no statistical differences between the groups.
The demographic data, ethnicity, disease duration, and mean age at disease onset in our study were in line with other rare reports of atypical patterns of FFA. 4,6 However, the proportion of each atypical pattern varies widely between studies. Kanti et al., in a series of 490 patients with FFA, found 32% with the ophiasic pattern (called the ''band-like'' pattern). 8 Rossi et al. described this pattern in 6.1% of their patients with FFA. These authors also found 12.2% of patients with the male pattern and 2.0% with the plaque pattern (called the ''headdress'' pattern). 6 Recently, Goldman et al. described a case of fibrosing frontal alopecia with a symmetrical 'epsilon-shaped' alopecia pattern, which mimicked traction alopecia with symmetrical alopecia in the temporal area. 9 In the present series, the plaque pattern, which would be the localized form of the disease, seems to have the best prognosis and the slowest evolution. These patients did not have manifestations such as facial papules and lichen planus pigmentosus, but there was no statistical difference from the other patterns.
The ophiasic pattern had a worse prognosis, and the male pattern had an intermediate prognosis. Both types showed the presence of facial papules, lichen planus pigmentosus and eyebrow alopecia, resembling cases of more aggressive disease.
The small number of patients, due to the lower incidence of atypical cases, is a limitation of the present study.
The study of these unusual presentations can help to improve the diagnosis and understand the prognosis of FFA.

Financial support
None declared.

Authors' contributions
Vanessa B Rocha: Statistical analysis; approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; intellectual participation in propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript. Carla Jorge Machado: Statistical analysis; approval of the final version of the manuscript; design and planning of the study; collection, analysis, and interpretation of data; effective participation in research orientation; critical review of the manuscript.
Leticia A. Contin: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.