Familial frontal fibrosing alopecia: A cross-sectional study of 20 cases from nine families

Australas J Dermatol. 2019 May;60(2):e113-e118. doi: 10.1111/ajd.12951. Epub 2018 Nov 15.

Abstract

Background/objectives: Frontal fibrosing alopecia (FFA) is a scarring alopecia whose prevalence is increasing. The pathogenesis of this disease is not well known. Genetic, environmental, hormonal and autoimmunity related factors have been considered; however, only a few cases of familial frontal fibrosing alopecia have been reported.

Material and methods: A cross-sectional study was performed at University Hospital in Granada (Spain). Twenty patients with frontal fibrosing alopecia belonging to nine different families were included, and clinical and dermoscopic features were analysed.

Results: Overall, 90% of the patients studied were women (mean age 61.4 years). About 50% of the patients had grade II frontal fibrosing alopecia at the time of diagnosis, whilst 35% had grades III or V. Mean recession was 2.83 cm in the frontal area and 1.99 cm in the temporo-parietal area. Daughters presented a shorter recession area and earlier debut of the disease than mothers. Androgenetic alopecia was found in only two patients (10%). The dermoscopic signs most commonly found were perifollicular erythema (85%), hyperkeratosis (85%), and absence of vellus hair in the hairline (78.9%).

Conclusion: This study adds to the growing evidence that there is a genetic component to frontal fibrosing alopecia. The clinical pattern of frontal fibrosing alopecia was not different from that found in non-familial cases, but the debut of the disease in daughters of mothers with frontal fibrosing alopecia may be earlier.

Keywords: cicatricial alopecia; familial; frontal fibrosing alopecia; hair; scarring alopecia.

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Alopecia / classification
  • Alopecia / genetics*
  • Alopecia / pathology
  • Atrophy
  • Cross-Sectional Studies
  • Dermoscopy
  • Erythema / complications
  • Female
  • Fibrosis
  • Genetic Predisposition to Disease
  • Hair Follicle / pathology
  • Humans
  • Keratosis / complications
  • Lymphocytes / pathology
  • Male
  • Middle Aged
  • Sebaceous Glands / pathology
  • Sex Distribution
  • Spain
  • White People