Unusual patterns of presentation of frontal fibrosing alopecia: A clinical and trichoscopic analysis of 98 patients

      To the Editor: Frontal fibrosing alopecia (FFA) is a primary lymphocytic scarring alopecia that mainly affects postmenopausal women.
      • Kossard S.
      Postmenopausal frontal fibrosing alopecia: scarring alopecia in a pattern distribution.
      In the past 2 decades, an increasing number of cases have been reported.
      • Martínez-Pérez M.
      • Churruca-Grijelmo M.
      Frontal fibrosing alopecia: an update on epidemiology and treatment.
      Recognizing FFA may be a challenge when it manifests with unusual features.
      The aim of this study was to evaluate clinical and trichoscopic features of FFA in a large series of patients.
      We performed a retrospective, monocentric study of trichoscopic images of 98 patients (4 men and 94 women) with FFA seen at our Dermatologic Clinic between 2003 and 2016. The diagnosis of FFA was based on clinical (symmetric frontal or frontotemporal hairline recession) and typical trichoscopic findings (absence of follicular openings, lone hairs, and single-hair pilosebaceous units). All patients were white, without familial history of FFA. Two patients were premenopausal at the onset.
      In our case series in 80 patients, FFA manifested with typical clinical features, whereas in 18 postmenopausal patients (18.4%), we observed unusual patterns of disease. In particular, 12 women presented with marked and symmetric recession of frontotemporal hairlines, with a peculiar sparing of the paramedian frontal hairline, mimicking male pattern androgenetic alopecia (AGA). We named this peculiar clinical presentation “AGA-like pattern” (Fig 1, A and C). Trichoscopic examination revealed typical findings of FFA (Fig 1, B and D). Moreover, in 2 patients we observed bilateral oval patches of alopecia in the temporal regions, with peculiar sparing of a band of temporal hairlines, in addition to typical recession of frontal hairline (Fig 2, A). Trichoscopy revealed typical findings of FFA, leading to a diagnosis of FFA in a peculiar clinical pattern that we called “cockade-like pattern” (Fig 2, B). Continuous involvement of the hairline from frontal to occipital regions was present in 6 other patients (Fig 2, C). Trichoscopic examination revealed in 4 of 6 cases typical findings of FFA at all areas affected (Fig 2, D). We termed this clinical finding “ophiasis-like pattern.” Instead, in 2 of 6 cases, trichoscopic examination revealed signs of alopecia areata at the occipital region, whereas typical features of FFA were present at the frontotemporal and retroauricolar regions.
      Figure thumbnail gr1
      Fig 1Frontal fibrosing alopecia (male androgenetic alopecia-like pattern) (A and C). Two cases of female patients had marked and symmetric recession of frontotemporal hairlines, with a peculiar sparing of the paramedian part of frontal hairline and the presence of bandlike atrophic areas with some terminal hairs in scarring outcome (black arrows). Frontal fibrosing alopecia (male androgenetic alopecia-like pattern) (B and D). Trichoscopic examination: scarring white patches with lack of follicular openings (green arrows), mild perifollicular scaling and erythema (red arrows), and some terminal hairs and single-hair pilosebaceous units (black arrows).
      Figure thumbnail gr2
      Fig 2Frontal fibrosing alopecia (cockade-like pattern) (A). A 63-year-old female patient with symmetric oval patches of alopecia, with a peculiar thin band of temporal hairline sparing. Loss of eyebrows was also present. Frontal fibrosing alopecia (cockade-like pattern) (B). Trichoscopic examination: absence of follicular openings (green arrow), mild perifollicular scaling and erythema (red arrow), and some terminal hairs and single-hair pilosebaceous units (black arrows). Frontal fibrosing alopecia (ophiasis-like pattern) (C). A 56-year-old female patient with irregular alopecic area involving from frontal to occipital area, mimicking alopecia areata. Frontal fibrosing alopecia (D). Trichoscopic examination: absence of follicular openings (green arrow), mild perifollicular scaling (red arrow), and some terminal hairs and single-hair pilosebaceous units (black arrows).
      Trichoscopy is a fast, noninvasive, and cost-efficient technique that is very useful in the diagnosis of hair and scalp diseases.
      • Miteva M.
      • Tosti A.
      Hair and scalp dermatoscopy.
      In particular, its efficacy has been demonstrated to diagnose and differentiate alopecia areata from other patchy alopecia and to diagnose early AGA.
      • Miteva M.
      • Tosti A.
      Hair and scalp dermatoscopy.
      Recently, Fernández-Crehuet et al
      • Fernández-Crehuet P.
      • Rodrigues-Barata A.R.
      • Vañó-Galván S.
      • et al.
      Trichoscopic features of frontal fibrosing alopecia: results in 249 patients.
      considered trichoscopy a valuable tool also in the diagnosis of FFA. The main trichoscopic features of FFA are absence of follicular openings, scarring white patches, minor perifollicular scaling, perifollicular erythema, and lone hairs.
      • Fernández-Crehuet P.
      • Rodrigues-Barata A.R.
      • Vañó-Galván S.
      • et al.
      Trichoscopic features of frontal fibrosing alopecia: results in 249 patients.
      Vaňó-Galván et al
      • Vañó-Galván S.
      • Molina-Ruiz A.M.
      • Serrano-Falcón C.
      • et al.
      Frontal fibrosing alopecia: a multicenter review of 355 patients.
      reported that typical dermoscopic features could substitute for biopsy in patients with typical FFA.
      We observed in a large case series that 18.4% of patients affected by FFA had unusual clinical findings; we could identify 3 patterns: (1) AGA-like pattern; (2) cockade-like pattern; and (3) ophiasis-like pattern. Trichoscopy revealed in all patients affected by FFA, both with typical and unusual clinical patterns, the same repetitive findings, namely the absence of follicular openings, scattered lone hairs, and single-hair pilosebaceous units at margins, also suggesting that when clinical presentation is not conclusive for the diagnosis of FFA, trichoscopy may be a very useful tool in the differential diagnosis.

      References

        • Kossard S.
        Postmenopausal frontal fibrosing alopecia: scarring alopecia in a pattern distribution.
        Arch Dermatol. 1994; 130: 770-774
        • Martínez-Pérez M.
        • Churruca-Grijelmo M.
        Frontal fibrosing alopecia: an update on epidemiology and treatment.
        Actas Dermosifiliogr. 2015; 106: 757-758
        • Miteva M.
        • Tosti A.
        Hair and scalp dermatoscopy.
        J Am Acad Dermatol. 2012; 67: 1040-1048
        • Fernández-Crehuet P.
        • Rodrigues-Barata A.R.
        • Vañó-Galván S.
        • et al.
        Trichoscopic features of frontal fibrosing alopecia: results in 249 patients.
        J Am Acad Dermatol. 2015; 72: 357-359
        • Vañó-Galván S.
        • Molina-Ruiz A.M.
        • Serrano-Falcón C.
        • et al.
        Frontal fibrosing alopecia: a multicenter review of 355 patients.
        J Am Acad Dermatol. 2014; 70: 670-678

      Linked Article

      • Acknowledging the pseudo “fringe sign” in frontal fibrosing alopecia has diagnostic and prognostic implications
        Journal of the American Academy of DermatologyVol. 78Issue 1
        • In Brief
          To the Editor: A frontal band of scarring alopecia with eyebrow involvement in postmenopausal women (ie, frontal fibrosing alopecia [FFA]) evolved from a “recently described disease” to the status of “a growing epidemic” in less than 2 decades. Since the first report by Kossard,1 additional features and variants not contemplated in the original description have been incorporated into the clinical picture. For such, I read with great interest the paper by Rossi et al reporting 3 unusual clinical patterns of FFA.
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