Frontal Fibrosing Alopecia: Is There a Link in Relatives?

Abstract

Frontal fibrosing alopecia (FFA) is an acquired primary lymphocytic cicatricial alopecia characterized by frontotemporal hairline recession, leading to scarring alopecia with a band-like distribution. Prevalence is increasing worldwide, being the most frequent cause of primary scarring alopecia. The natural history of this condition is variable; however, slow progression with spontaneous remission is the most frequent reported outcome. The etiopathogenesis of FFA remains to be elucidated; numerous hypotheses concerning hormonal effects, environmental factors, and genetic predisposition have been proposed. Special interest on genetic basis has emerged since the first familial case was reported. Only a few more familial cases have been published. We report 6 additional cases of female patients with familial FFA (F-FFA) from 3 different families. Sixty-six percent had a family history of autoimmune disease in first-degree relatives; these same patients had a personal history of autoimmune disease. The families described in this cohort study plus the personal and family history of autoimmune disease, as well as the recently described involved genomic loci; reinforced the hypothesis of this disease being genetic. It is important to consider studying this entity since there are scarce data regarding familial cases and this might give us a better insight toward understanding its pathogenesis.

Frontiers in Lichen Planopilaris and Frontal Fibrosing Alopecia Research: Pathobiology Progress and Translational Horizons

“…Human eHFSCs seem to have yet another Achilles heel: their vulnerability to undergo pathological epithelial-mesenchymal transition (EMT). Both LPP and FFA are typically associated with scarring/fibrosis (Doche et al 2020b;Harries et al 2018;Ocampo-Garza et al 2021). This cannot be credibly explained by the apoptosis-induced depletion of the epithelial SCs and their progeny, since as the loss of epithelial cells induces tissue atrophy, but not fibrosis.…”

A review of isotretinoin in the treatment of frontal fibrosing alopecia

“…7,[44][45][46] Moreover, a theory suggests that FFA may have a genetic basis, as the condition has been observed affecting multiple members of the same family. 47 Tziotzios et al proposed that FFA may be inherited in an autosomal dominant manner with incomplete penetrance. 46 However, the fact that FFA typically develops later in life suggests that environmental factors may also play a role in the development of the disease.…”

“…Moreover, a theory suggests that FFA may have a genetic basis, as the condition has been observed affecting multiple members of the same family 47 . Tziotzios et al.…”

Frontal Fibrosing Alopecia and Reproductive Health: Assessing the Role of Sex Hormones in Disease Development

“…Genetic vulnerability to FFA is also evident, as human leucocyte antigen (HLA) profiling studies have identified two susceptible haplotypes (C17:01:01:02/B42:01:01 and C07:02:01:03/B07:02:01:01) in familial cases profiling studies [8]. A second study supports the genetic etiology by demonstrating that a high percentage (66%) of FFA patients have both a personal history and a first-degree family history of autoimmune disease [9]. Moreover, hypothyroidism due to autoimmune thyroiditis is associated with up to 30% of patients with FFA [10].…”