Peripilar “Guttate” Hypopigmentation of the Scalp and Idiopathic Guttate Hypomelanosis in Frontal Fibrosing Alopecia

Abstract

Frontal fibrosing alopecia (FFA) is characterized by progressive band-like scarring alopecia involving the frontotemporal hairline and limbs and irreversible alopecia of the eyebrows. Two opposite types of pigmentary alterations have been reported in FFA: hyper- and hypopigmentation. Here, we report a 57-year-old Hispanic female with FFA who presented with peripilar hypopigmentation and idiopathic guttate hypomelanosis on the upper extremities. To our knowledge, this is the first report of peripilar white halos in a patient of a White Hispanic origin. Upon review of other previous publications on trichoscopy of FFA, this pattern has not been detected. The common pattern of focal absence of melanocytes and melanin in both is another confirmation that FFA is associated with pigmentation abnormalities.

Established Facts

• Frontal fibrosing alopecia (FFA) is a type of lymphocyte-mediated cicatricial alopecia.

• Hypo- and hyperpigmentary alterations have been reported in FFA.

Novel Insights

• This is the first report of peripilar white halos described in a patient of White Hispanic origin with FFA.

• The coexistence of perifollicular hypopigmented halos and idiopathic guttate hypomelanosis in a patient with FFA confirms its association with pigmentation abnormalities.

Introduction

Frontal fibrosing alopecia (FFA) is characterized by progressive band-like scarring alopecia involving the frontotemporal hairline and sideburns and irreversible alopecia of the eyebrows, beard (in men), and limbs [1]. Two opposite types of pigmentary alterations have been reported in FFA (Table 1). (1) Hyperpigmentation: lichen planus pigmentosus (LPPigm) is a variant of lichen planus characterized by dark brown macules distributed in sun-exposed areas [1]. More than 50% of the FFA patients from African and Indian ethnic backgrounds have co-existent LPPigm [2]. (2) Hypopigmentation: a small histologic study by Lin et al. [3] demonstrated an association between hypopigmentation of the alopecic band highlighted under a Wood's lamp examination with decreased epidermal melanocyte numbers on histology. These results were not appreciated in lichen planopilaris cases where melanocyte counts were similar to those of controls. Furthermore, there are few reports on the coexistence of FFA and vitiligo [4]. A hypothesis was even proposed that the development of FFA on areas affect ed by vitiligo may indicate a common pathogenic background involving CD8+ cytotoxic lymphocytes [5]. We report peripilar hypopigmentation and idiopathic guttate hypomelanosis (IGH) in a White Hispanic female with FFA.

Table 1.

Pigmentary alterations reported in FFA (listed in alphabetical order)

First author [ref.], year	Study type	Subjects	Ethnicity	Associated pigmentary alteration
Banka [11], 2014	Cohort study	1	Caucasian origin	Vitiligo
Berliner [12], 2014	Case report	2	Hispanic origin	Lichen planus pigmentosus
Dlova [2], 2013	Case series	24	22 African origin 2 Indian origin	Lichen planus pigmentosus
Franco-Munoz [13], 2017	Case report	1	Caucasian origin	Lichen planus pigmentosus
Katoulis [14], 2016	Case report	2	Caucasian origin	Vitiligo
Lin [3], 2017	Case control	19	Not reported	Decreased melanocyte count
Lopez Pestana [15], 2015	Case series	8	Caucasian origin	Pigmented lentiginous macules
Miteva [4], 2011	Case series	4	3 Caucasian origin
1 Hispanic origin	Vitiligo
Mulinari-Brenner [1], 2017	Case report	1	Not reported	Lichen planus pigmentosus
Pirmez [16], 2016	Case report	37	33 Hispanic and 4 African origin	Lichen planus pigmentosus
Rao [17], 2014	Case report	1	Indian origin	Lichen planus pigmentosus
Tan [18], 2009	Case report	1	Not reported	Vitiligo
Vano-Galvan [19], 2014	Retrospective review	2	Caucasian origin	Vitiligo

Case Presentation

A 57-year-old female, Fitzpatrick skin type IV, presented with a few years' history of a receding hair line with involvement of the temples, sideburns, and eyebrows, accompanied by severe pruritus (Fig. 1a). At the time of presentation, she was only on topical clobetasol solution but had received intralesional triamcinolone injections in the past. Her history was significant for white spots on the upper extremities for many years (Fig. 1b). Clinical examination showed a relatively preserved, 1-cm reduced frontal hair line with a patchy decreased hair density due to follicular drop out and absence of vellus hairs, more pronounced on the temples and sideburns. The upper extremities revealed a symmetric distribution of scattered white macules. Trichoscopy of the frontotemporal scalp revealed a loss of vellus hairs and a 0.2-to-0.3-mm peripilar annular hypopigmented/white halo around most terminal hairs (Fig. 2). No peripilar casts were detected. Dermoscopy of the white macules on the extremities demonstrated the “cloudy sky-like” pattern, typical of IGH [6] (Fig. 1b). Scalp biopsy was consistent with lymphocytic cicatricial alopecia in the current setting with FFA.

Fig. 1.

a, b Coexistence of IGH of the upper extremities with FFA of the frontotemporal hairline in a 57-year-old Hispanic female. The image was obtained with a Fotofinder dermatoscope (original magnification ×20).

Fig. 2.

Frontal fibrosing alopecia. Dermatoscopic findings revealed peripilar white halos around emerging hairs of the frontal line. Note the absence of vellum hairs. The image was obtained with a Fotofinder dermatoscope (original magnification ×20).

Discussion

To our knowledge, this is the first report of peripilar white halos in a patient of White Hispanic origin. In this population, an active area of cicatricial alopecia typically shows peripilar erythema and casts [7]. We and others have previously published on trichoscopy of FFA and have not detected this pattern [8, 9]. A peripilar white/gray halo is considered a specific and sensitive finding for the diagnosis of central centrifugal cicatricial alopecia [7]. However, the halo in central centrifugal cicatricial alopecia is usually around groups of 2–3 hairs emerging from the same ostium and surrounds vellus hairs, which disappear first in FFA [7]. A possible explanation for this follicular-bound hypopigmentation in FFA can be the presence of persistent lichenoid inflammation and respective cytokine release at the isthmus level and below, leading to a reduction of melanocyte stem cells. In the follicular melanogenesis, activation of a melanocyte stem cell leads to the development of melanocyte progenitors and differentiated melanocytes, which produce melanin. Packets of melanin are transferred to adjacent keratinocytes, which confers color to the hair shaft and the outer root sheath that transitions into the interfollicular epithelium at the infundibulum [10]. The hypopigmented halos in our patient were not a sign of postinflammatory hypopigmentation because the patient's disease was still active given her significant itching.

In conclusion, our case is unique due to the unusual pattern of perifollicular hypopigmented halos observed in white skin and its association with IGH. We do not know if the coexistence of IGH and FFA is coincidental but we believe that the common pattern of focal absence of melanocytes and melanin in both is another confirmation that FFA is associated with pigmentation abnormalities.

Statement of Ethics

The patient presented in this paper gave her informed consent for the above presentation.

Disclosure Statement

The authors declare that they have no conflicts of interest and no funding sources to disclose.