Frontal Fibrosing Alopecia and Concomitant Lichen Planus Pigmentosus: A Case Series of Seven African American Women

April 2018 | Volume 17 | Issue 4 | Original Article | 397 | Copyright © 2018

Laura N. Uwakwe MD, Leah A. Cardwell MD, Emily H. Dothard MD, Bernice I. Baroudi BS, and Amy J. McMichael MD

Department of Dermatology, Wake Forest School of Medicine, Winston-Salem, NC

Abstract

The association of frontal fibrosing alopecia (FFA) and lichen planus pigmentosus (LPPigm) is rare. Prior reports suggest that FFA and LPPigm are on the same spectrum of disease, and a diagnosis of LPPigm may predict the future development of FFA. We aim to further characterize the association between FFA and LPPigm by reviewing the clinical cases of seven African American women. Seven patients with FFA were diagnosed clinically by recession of frontotemporal hairline and confirmed by histopathologic examination showing lymphocyte-mediated cicatricial alopecia. LPPigm was diagnosed by clinical evaluation alone based on the characteristic morphology, color, and distribution of the lesions. It is difficult to distinguish whether halted progression of FFA was due to the success of the treatment regimen or spontaneous stabilization of disease over time. Our case series supports the theory that FFA and LPPigm likely exist on the same spectrum of disease. Our observations demonstrate a likely positive correlation between FFA and LPPigm.

J Drugs Dermatol. 2018;17(4):397-400.

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BACKGROUND

Frontal brosing alopecia (FFA) is a lymphocyte-mediated cicatricial alopecia, and considered a variant of lichen planopilaris. It is characterized by progressive recession of the frontotemporal hairline, often with loss of the eyebrows.1 While FFA typically affects postmenopausal, Caucasian women, recent case reports have documented the condition in premenopausal women, various ethnicities, and rarely in men.2,3 In African-American patients, the age of onset tends to be earlier; follicular hyperpigmentation may be pronounced; and typical scarring alopecia characteristics such as erythema and follicular papules are uncommon.4 Lichen planus pigmentosus (LPPigm) is an uncommon variant of lichen planus which primarily affects young to middle aged individuals with Fitzpatrick skin types III-V. The condition is characterized by slate gray to brown macules and patches, on sun-exposed or exural areas, in diffuse or reticulated patterns. LPPigm generally has an insidious onset with a chronic clinical course of exacerbations and remissions.5 FFA with concomitant LPPigm is rare with just a few reports in the literature.6-8 We report the cases of seven African American women with FFA and concomitant LPPigm to further characterize the association between these conditions. A retrospective review of medical records, clinical photos, and histopathology reports of seven African American women with FFA and LPPigm was conducted at Wake Forest Baptist Health Dermatology Clinic in Winston-Salem, North Carolina, USA.

REPORT OF CASES

Seven patients presented to the dermatologist due to frontotemporal hair loss. FFA was diagnosed clinically by recession of frontotemporal hairline and confirmed by histopathologic examination showing lymphocyte-mediated cicatricial alopecia. Four of the seven patients (57%) were diagnosed with FFA prior to the onset of LPPigm; the mean lag interval between FFA and LPPigm development was 2.25 years. Three out of seven patients (43%) were diagnosed with LPPigm prior to the onset of FFA, with a mean lag interval between LPPigm and FFA development of 4.3 years. One of the four patients with FFA preceding LPPigm diagnosis had eyebrow loss at the time of FFA diagnosis, one had no eyebrow loss, and two had eyebrow loss within 5 to 7 years after FFA diagnosis. Two of the three patients with LPPigm preceding FFA diagnosis had eyebrow loss within 5 to 7 years after the diagnosis of LPPigm. Six of the patients endorsed pruritus and/or burning sensation in the scalp prior to frontotemporal hair loss. Three of the four patients with FFA preceding LPPigm diagnosis, who were treated with a combination of oral hydroxychloroquine 200-400mg daily and topical and/or intralesional corticosteroid injections, demonstrated clinically slowed FFA progression. One of four patients with FFA preceding LPPigm diagnosis experienced progression of FFA while on a combination of topical and intralesional corticosteroids and hydroxychloroquine 400mg daily; FFA progression was halted by the addition of dutasteride 0.5mg to the treatment regimen.

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