Patchy Frontal Fibrosing Alopecia: Description of an Incomplete Clinical Presentation

Abstract

We describe 6 incomplete cases of frontal fibrosing alopecia (FFA). All patients were postmenopausal women. Their average age was 60 years; 2 of the women were Caucasian, and the remaining 4 women were of a mixed ethnicity. The disease duration ranged from 3 to 17 years. The patients' lesions presented as irregular alopecic plaques in the frontal and/or temporal regions, with few signs of perifollicular inflammation, slow progression, and progressive loss of the vellus hair in the frontal area. Loss of the eyebrows, facial hyperpigmentation, and loss of the body hair were observed in 3 patients, 1 patient, and 2 patients, respectively. Localized pruritus and dysesthesia were reported in 5 patients and 3 patients, respectively. Dermatoscopic examination indicated the absence of viable ostia at the centers and edges of the lesions and the absence of peripilar erythema; moreover, peripilar hyperkeratosis was less intense than it would be expected in classical cases of lichen planopilaris (LPP) and FFA. Histological examination revealed that all cases featured the presence of a perifollicular lichenoid infiltrate in the infundibulum and isthmus as well as concentric areas of fibrosis. All samples were subjected to direct immunofluorescence (DIF), which produced negative results. FFA has been recently diagnosed and is considered a subtype of LPP. FFA can present with incomplete clinical manifestations, a histology similar to LPP, and negative DIF findings. The correct diagnosis of FFA-associated lesions is critical for providing appropriate treatment, developing prognostic definitions, and excluding other types of scarring and nonscarring alopecia. The diagnosis of incomplete FFA is essential to avoid diagnostic and therapeutic errors.

A Novel Atypical Presentation of Frontal Fibrosing Alopecia Involving the Frontoparietal Scalp

“…Three unusual presentations have been identified as well, including (1) androgenetic alopecia-like pattern, (2) cockade-like pattern, and (3) ophiasis-like pattern [9]. FFA usually presents with symmetric bilateral involvement, although an asymmetrical irregular presentation has also been reported [10] and observed by one of us as an asymmetric cockade-like pattern (authors' unpublished data). Herein, we report an unusual presentation of FFA in an "upsilon" pattern not previously described.…”

“…As mentioned, there have been a number of reports of atypical FFA presentations that do not fit the classic description. Atypical presentations of FFA differ from the classic frontotemporal FFA by the distribution of alopecia but maintain the same trichoscopic and histologic findings [8][9][10]. Regarding prognosis, among the classic types the "pseudo-fringe" pattern has been associated with less progression, whereas the majority of treated patients with the "zig-zag" pattern progressed despite treatment [8].…”

Clinical Characteristics of Frontal Fibrosing Alopecia in Brazil: A Series of 59 Patients

“…The clinical patterns (based upon hair line recession) [14][15][16] (Fig. 1) were defined through patients' photos by a single observer.…”

Frontal fibrosing alopecia: a disease that remains enigmatic

“…Continuous involvement of the hairline from frontal to occipital regions -ophiasis-like pattern -was present in 6 women. Contin reported six FFA cases with incomplete presentations, namely unevenly distributed irregular alopecic plaques, the presence of lonely hairs, the absence of inflammatory peripilar signs, and slow progression [42].…”

“…Continuous involvement of the hairline from frontal to occipital regions – ophiasis-like pattern – was present in 6 women. Contin et al reported six FFA cases with incomplete presentations, namely unevenly distributed irregular alopecic plaques, the presence of lonely hairs, the absence of inflammatory peripilar signs, and slow progression [ 42 ]. For that matter, on this point it should be added that FFA can be non-inflammatory on clinical presentation in locations such as the sideburns, eyebrows, trunk, and limbs.…”