Frontal fibrosing alopecia in association with Sjögren's syndrome: more than a simple coincidence

Furlan, Karina; Kakizaki, Priscila; Chartuni, Juliana Cabral Nunes; Valente, Neusa Yuriko Sakai

doi:10.1590/abd1806-4841.20164526

Cited by 14 publications

(13 citation statements)

References 10 publications

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“…Many studies have noted the apparent association of FFA with autoimmune diseases, including hypothyroidism and Sjögren’s syndrome, among others [3, 24, 29], and similar findings were present in our study population. Rosacea, which has recently been reported to have a prevalence of 34% ( n = 35) [30], was only seen in 4% of our patients.…”

Section: Discussionsupporting

confidence: 91%

Facial and Extrafacial Lesions in an Ethnically Diverse Series of 91 Patients with Frontal Fibrosing Alopecia Followed at a Single Center

2018

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Background: Various facial and extrafacial lesions have been reported in frontal fibrosing alopecia (FFA). Facial papules have been associated with worse prognosis. Objectives: We sought to detect the prevalence of facial and extrafacial lesions and to analyze their relation to demographic and clinical variables in a large and ethnically diverse series of patients with FFA. Methods: Charts of patients diagnosed with FFA between January 1, 2015, and December 31, 2017, at the Department of Dermatology, University of Miami, were reviewed retrospectively. Results: 91 patients (87 women and 4 men) met inclusion criteria: 45% (n = 41) were of Hispanic/Latino ethnicity, and 34% (n = 30) were premenopausal. Facial papules were most commonly detected (41% among Hispanic/Latino patients). Significant associations were found between: (1) Hispanic/Latino ethnicity and any FFA-associated facial lesions, facial papules alone, or lichen planus pigmentosus alone, as well as premenopausal status; (2) any FFA-associated facial lesions or facial papules alone and premenopausal status; and (3) Hispanic/Latino ethnicity and simultaneous presence of facial and extrafacial lesions. Conclusions: There is a significant association among Hispanic/Latino ethnicity, facial papules, and premenopausal status, which may portend a susceptibility to severer disease and prompt early and aggressive treatment in this group.

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Section: Discussionsupporting

confidence: 91%

Facial and Extrafacial Lesions in an Ethnically Diverse Series of 91 Patients with Frontal Fibrosing Alopecia Followed at a Single Center

2018

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“…We found a strong association between FFA and LPP, which were associated in almost 1/4 of the patients, suggesting that FFA represents a clinical variant of LPP, with an autoimmune pathogenesis. According to literature, 3,[12][13][14][15][16][17] we found a significant prevalence of other autoimmune diseases (50.8%) in our patients and a high percentage of patients with atopic background (35.4%), suggesting a possible personal predisposition to immune system disorders.…”

Section: Discussionsupporting

confidence: 64%

Frontal fibrosing alopecia: a case series of 65 patients seen in a single Italian centre

Starace

Brandi

Alessandrini

et al. 2018

Acad Dermatol Venereol

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Background Frontal fibrosing alopecia (FFA) is increasing in prevalence worldwide and several series from different countries have been published, in order to detect possible causes of the disease outbreak. Objective To analyze the epidemiological, clinical and trichoscopic features of FFA seen in an Italian Dermatology Unit. Methods Clinical, trichoscopy, histopathology and medical details of all patients were compared with literature and general population data. Results From 2005 to 2017, we diagnosed FFA in 65 Caucasian females, with the number of diagnoses per year progressively rising. Premenopausal onset was detected in 21%. Frontal hair line recession was associated with parietal involvement in 80% of cases, occipital in 12.3% and eyebrows alopecia in 86.1%. In six cases, eyebrow alopecia preceded hair loss. Non‐inflammatory facial papules were detected in 1/3 of the patients. Itching was reported by 2/3 of the patients and was indicative of disease progression. Trichoscopy showed empty follicles/yellow dots, absence of follicular ostia, mild follicular hyperkeratosis, perifollicular erythema and ‘lonely hair’. Scalp lichen plano‐pilaris was seen in 15 patients, female pattern hair loss in 22. Therapy included short‐term treatment with systemic or intralesional corticosteroids followed by therapy with 5α‐reductase inhibitors of hydroxychloroquine and topical drugs. Arrest of FFA progression was seen in 75% of the patients, while 16 experienced worsening of the hairline despite therapy. Conclusions Frontal fibrosing alopecia not rarely starts before menopause, loss of the eyebrows can be the first sign of the disease, about 50% of the patients have other autoimmune conditions, FFA severity is not related to its duration, itching, follicular hyperkeratosis and erythema at trichoscopy are signs of disease progression, 25% of the patients show progression of hairline recession despite therapy.

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“…Numerous associations of cutaneous and systemic diseases with FFA have been reported previously, including, among others, rheumatoid arthritis, vitiligo, Sjogren's syndrome, systemic and discoid lupus erythematosus, pemphigoid, alopecia areata, Crohn's disease, psoriasis and multiple sclerosis . Coexisting of autoimmune and immune‐mediated diseases in the same individuals is not rare . A reported association of FFA with rosacea could not be confirmed in this sample, as the observed prevalence of rosacea was lower than in the general population .…”

Section: Discussionmentioning

confidence: 55%

“…Dermatological and systemic autoimmune disorders were reported in 7% of the women in this sample. Numerous associations of cutaneous and systemic diseases with FFA have been reported previously, including, among others, rheumatoid arthritis, vitiligo, Sjogren's syndrome, systemic and discoid lupus erythematosus, pemphigoid, alopecia areata, Crohn's disease, psoriasis and multiple sclerosis . Coexisting of autoimmune and immune‐mediated diseases in the same individuals is not rare .…”

Section: Discussionmentioning

confidence: 91%

Frontal fibrosing alopecia: demographic and clinical characteristics of 490 cases

Kanti

Constantinou

Reygagne

et al. 2019

Acad Dermatol Venereol

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Background Frontal fibrosing alopecia (FFA) is a cicatricial alopecia mostly affecting the frontotemporal hairline. Its aetiology and associated factors remain unclear.Objective and methods An observational, cross-sectional and descriptive study was conducted in France and Germany to identify demographic and health characteristics associated with the severity of FFA. ResultsOf 490 included patients, 95% were female, of which 84% were postmenopausal. Age at onset of FFA symptoms ranged between 15 and 89 years, but diagnosis was frequently delayed up to 24 years. Lichen Planopilaris Activity Index scores were low (median 1.8, IQR 1.0 to 3.5). Thyroid function disorders were reported in 13% of men and 35% of women. Abnormal blood lipid levels were found in 42% of tested men and 47% of women. In the bivariate analyses, LPPAI scores were negatively correlated with abnormal testosterone (r s = À0.775) and oestrogen values (r s = À0.664), regular use of face cleaning products (r s = À0.465), hair colourants (r s = À0.679) and hairspray (r s = À0.500).Conclusions The most common comorbidity was thyroid disease, with proportions higher than in the European population, possibly reflecting a role of thyroid hormones in FFA pathogenesis. The association of abnormal testosterone and oestrogen values with lesser disease activity needs to be explored in further studies. Our correlation analyses do not support a role of leave-on cosmetic products in the pathophysiology of FFA.

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Frontal fibrosing alopecia in association with Sjögren's syndrome: more than a simple coincidence

Cited by 14 publications

References 10 publications

Facial and Extrafacial Lesions in an Ethnically Diverse Series of 91 Patients with Frontal Fibrosing Alopecia Followed at a Single Center

Facial and Extrafacial Lesions in an Ethnically Diverse Series of 91 Patients with Frontal Fibrosing Alopecia Followed at a Single Center

Frontal fibrosing alopecia: a case series of 65 patients seen in a single Italian centre

Frontal fibrosing alopecia: demographic and clinical characteristics of 490 cases

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