Skin of Color

Frontal Fibrosing Alopecia: Cutaneous Associations in Women With Skin of Color

In Collaboration with the Skin of Color Society

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Frontal fibrosing alopecia (FFA) was first described as a progressive recession of the frontal hairline in postmenopausal women. Since its initial description, recognition and understanding of FFA has expanded. The condition is now defined as a patterned, symmetric, frontotemporal scarring alopecia that is considered to be histopathologically indistinguishable from lichen planopilaris. Numerous case reports and series have suggested clinical variants of and associations with FFA. In addition to reviewing the literature on FFA’s associations, this article includes a case series of 5 women with skin of color (Hispanic and black) who presented with various cutaneous findings in association with FFA, including lichen planus pigmentosus (LPP), facial papules, and eyebrow loss. Recognition of the conditions that can occur in association with FFA in individuals with skin of color is important in further expanding our knowledge and understanding of FFA as a disease entity.

Practice Points

  • Frontal fibrosing alopecia (FFA) is associated with lichen planus pigmentosus, especially in patients with skin of color.
  • Patients with FFA should be evaluated for additional cutaneous features including facial papules, glabellar red dots, and depressed frontal veins.


 

References

Frontal fibrosing alopecia (FFA) has been reported in association with lichen planus pigmentosus (LPP) and facial papules.1-3 Lichen planus pigmentosus is a variant of lichen planus that causes hyperpigmentation of the face, neck, and/or intertriginous areas that may be useful as a clinical indicator in the development of FFA.1 Facial papules in association with FFA are secondary to fibrosed vellus hairs.2,3 Currently, reports of concomitant FFA, LPP, and facial papules in women with skin of color are limited in the literature. This case series includes 5 women of color (Hispanic and black) who presented to our clinic with FFA and various cutaneous associations. A review of the current literature on cutaneous associations of FFA also is provided.

Case Reports

Patient 1
A 50-year-old Hispanic woman who was previously presumed to have melasma by an outside physician presented with pruritus of the scalp and eyebrows of 1 month’s duration. Physical examination revealed decreased frontal scalp hair density with perifollicular erythema and scale with thinning of the lateral eyebrows. Hyperpigmented coalesced macules (Figure 1A) and erythematous perifollicular papules were noted along the temples and on the perioral skin. Depressed forehead and temporal veins also were noted (Figure 1B). A biopsy of the scalp demonstrated perifollicular and perivascular lymphocytic inflammation and fibrosed hair follicles, and a biopsy of the perioral skin demonstrated perivascular lymphocytic inflammation with melanophages in the papillary dermis. A diagnosis of FFA with LPP was established with these biopsies.

Figure1

Figure 1. Frontal fibrosing alopecia with hyperpigmented coalesced macules around the mouth (A). Perifollicular papules on the temples (black arrow), erythematous perifollicular papules at the frontal hairline (blue arrow), and depressed veins on the forehead and temples (yellow arrows) also were noted (B).

Patient 2
A 61-year-old black woman presented with asymptomatic hair loss along the frontal hairline for an unknown duration. On physical examination the frontal scalp and lateral eyebrows demonstrated decreased hair density with loss of follicular ostia. Fine, flesh-colored, monomorphic papules were scattered along the forehead and temples, and ill-defined brown pigmentation was present along the forehead, temples, and cheeks. Biopsy of the frontal scalp demonstrated patchy lichenoid inflammation with decreased number of follicles with replacement by follicular scars, confirming the diagnosis of FFA.

Patient 3
A 47-year-old Hispanic woman presented with hair loss of the frontal scalp and bilateral eyebrows with associated burning of 2 years’ duration. Physical examination demonstrated recession of the frontotemporal hairline with scattered lone hairs and thinning of the eyebrows. Innumerable flesh-colored papules were present on the forehead and temples (Figure 2A). Glabellar and eyebrow erythema was noted (Figure 2B). Biopsy of the frontal scalp demonstrated decreased terminal anagen hair follicles with perifollicular lymphoid infiltrate and fibrosis, consistent with a diagnosis of FFA. The patient was started on oral hydroxychloroquine 400 mg once daily, and 3 months later hyperpigmentation of the forehead and perioral skin was noted. The patient reported that she had facial hyperpigmentation prior to starting hydroxychloroquine and declined a biopsy.

Figure2

Figure 2. Frontal fibrosing alopecia with recession of the temporal hairline with visible lone hairs (red arrow) and scattered flesh-colored papules on the temples (black arrows)(A). Glabellar and eyebrow erythema also was noted with flesh-colored papules on the forehead (black arrow)(B). The eyebrows were notably drawn in due to decreased hair density, and the central frontal hairline was recessed.

Patient 4
A 40-year-old black woman presented with brown pruritic macles of the face, neck, arms, and forearms of 4 years’ duration. She also reported hair loss on the frontal and occipital scalp, eyebrows, and arms. On physical examination, ill-defined brown macules and patches were noted on the neck (Figure 3), face, arms, and forearms. Decreased hair density was noted on the frontal and occipital scalp with follicular dropout and perifollicular hyperpigmentation. Biopsy of the scalp demonstrated perivascular lymphocytic inflammation with sparse anagen follicles and fibrous tracts, and biopsy of the neck revealed superficial perivascular inflammation with numerous melanophages in the upper dermis; these histopathologic findings were consistent with FFA and LPP, respectively.

Figure3

Figure 3. Diffuse and coalescing brown-gray macules and patches on the neck consistent with lichen planus pigmentosus.

Patient 5
A 46-year-old black woman with history of hair loss presented with hyperpigmentation of the face and neck of 2 years’ duration. On physical examination decreased hair density of the frontal and vertex scalp and lateral eyebrows was noted. Flesh-colored papules were noted on the forehead and cheeks, and confluent dark brown patches were present on the temples and neck. Three punch biopsies were performed. Biopsy of the scalp revealed lymphocytic inflammation with surrounding fibroplasia with overlapping features of FFA and central centrifugal cicatricial alopecia (Figure 4). Biopsy of the neck revealed vacuolar interface dermatitis. Additionally, biopsy of a facial papule revealed lichenoid inflammation involving a vellus hair follicle. Clinical and histopathological correlation confirmed the diagnosis of FFA with LPP and facial papules.

Figure4

Figure 4. Representative photograph demonstrating a diminished number of hair follicles with partial loss of sebaceous glands. There was perifollicular fibroplasia and interface inflammation along the basement membrane of the follicular epithelium with exocytosis of lymphocytes. Low-grade vacuolar alteration also was seen along the dermoepidermal junction (H&E, original magnification ×100).

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