Frontal fibrosing alopecia.

A 50-year-old woman presented with loss of scalp hair since two years. She had no other complaints. On examination, she had a single patch of cicatricial alopecia over the fronto-parietal area of scalp with marked recession of the frontal hairline [Figure 1]. No follicular papules on the lesion or elsewhere on the body were noted. Axillary and pubic hair was normal. Oral and genital mucosae were normal. She had been treated with intralesional triamcinolone injections, multiple topical glucocorticoid preparations, and tacrolimus ointment by various dermatologists, but without benefit. Laboratory tests including complete blood count, thyroid screen, and antinuclear factor were normal. We performed a biopsy from the lesion, which revealed absent hair follicles, hair follicles replaced by fibrous tracts and a chronic lymphocytic infiltrate surrounding the follicles suggesting a diagnosis of frontal fibrosing alopecia (FFA) [Figure 2].

Figure 1

Patchy hair loss over frontoparietal region of scalp

Figure 2

Histopathology showing absent hair follicles, hair follicles replaced with fibrous tract, and surrounding lymphocytic infiltrate (H and E, ×100)

FFA was first described by Kossard in 1994.[1] It is a form of cicatricial alopecia and commonly accepted as a variant of lichen planopilaris, based on shared features in clinical appearance and histopathology.[1] It is a variant of primary lymphocytic cicatricial alopecia, typically observed in postmenopausal women with a few cases reported in men.[2] Women with this condition typically present with the complaint of asymptomatic, progressive recession of their frontal hairline. Clinical examination shows symmetric, marginal alopecia along the frontal and frontal–parietal hairline. This form of scarring alopecia requires clinical pathologic correlation in order to make the diagnosis. Histopathologic examination of affected scalp shows a reduced number of hair follicles, which have been replaced by fibrous tracts, and a lymphocytic infiltrate in a lichenoid pattern about the infundibular, isthmus, and bulge portions of the remaining follicles. This perifollicular, lymphocytic, lichenoid inflammation is typical of lichen planopilaris, and FFA is considered to be a variant of lichen planopilaris.[3] Another lichen planopilaris variant, Piccardi–Lassueur–Graham–Little syndrome (PLGLS), is characterized by widespread follicular keratotic papules on the limbs and trunk and hair loss involving pubic hair, axillary hair, and the eyebrows. A spectrum of patients present with overlapping features of FFA and PLGLS and some familial cases of both entities have been reported.[1]

Additional evaluation of these patients may include a complete blood count, a thyroid function panel, androgens, erythrocyte sedimentation rate, antinuclear antibody, and antibodies to double-stranded DNA and extractable nuclear antigen. Most often, these laboratory investigations are normal.[3]

No treatment has been found to alter the course of FFA. Treatments that have been employed include oral prednisolone, chloroquine phosphate, moderate potency topical glucocorticoid preparations, intralesional glucocorticoids, topical retinoic acid, minoxidil 2% solution, oral isotretinoin, and ultramicronized griseofulvin but with minimal benefit. Hormone replacement therapy does not appear to alter the development or progression of this condition.[3]

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