Characterization of the inflammatory features of central centrifugal cicatricial alopecia

Abstract

Central centrifugal cicatricial alopecia (CCCA) is a scarring alopecia that primarily affects women of African descent. Although histopathological features of CCCA have been described, the pathophysiology of this disease remains unclear. To better understand the components of CCCA pathophysiology, we evaluated the composition of the inflammatory infiltrate, the distribution of Langerhans cells (LCs), and the relationship between fibrosis and perifollicular vessel distribution. Our data indicate that CCCA is associated with a CD4-predominant T-cell infiltrate with increased LCs extending into the lower hair follicle. Fibroplasia associated with follicular scarring displaces blood vessels away from the outer root sheath epithelium. These data indicate that CCCA is an inflammatory scarring alopecia with unique pathophysiologic features that differentiate it from other lymphocytic scarring processes.

SnapshotDx Quiz: May 2022

“…c. Central centrifugal cicatricial alopecia: Central centrifugal cicatricial alopecia (CCCA) is a type of scarring alopecia characterized by patches of permanent hair loss originating on the scalp vertex/ crown with centrifugal spread, associated with signs of inflammation (Flamm et al, 2020). CCCA is especially common among women of African descent (uncommon in men and children), with an average age of onset of 36 years (Malki et al, 2019).…”

“…The pathophysiology of CCCA is not well established (possibly owing to a shortage of studies in the literature); however, several studies have suggested that processes leading to premature desquamation of the inner root sheath (PDIRS) are critical in CCCA pathogenesis (Flamm et al, 2020). One group observed that mutations in the PADI3 gene may be correlated with CCCA (Malki et al, 2019).…”

Common causes of hair loss – clinical manifestations, trichoscopy and therapy

“…10). 71 Histopathological features associated with CCCA include premature desquamation of the inner root sheath (IRS), compound follicular structures with perifollicular fibrosis and naked hair shafts with a foreign‐body inflammatory reaction in fibrous streamers 72 …”

A niche in the spotlight: Could external factors critically disturb hair follicle homeostasis and contribute to inflammatory hair follicle diseases?

“…Flamm et al recently described increased numbers of LCs extending into the lower hair follicle in central centrifugal cicatricial alopecia. [ 58 ] Enhanced LC/T lymphocyte ratios were reported in lichen planopilaris compared to traction alopecia, [ 59 ] while a loss of LCs in interfollicular epidermis was described in association with hair follicle destruction in lichen planopilaris and folliculitis decalvans. [ 60 ] Perifollicular and intrafollicular LCs appear to be increased in hair follicle affected the frontal fibrosing alopecia, [ 61 ] but most investigations are limited to immunohistochemical stainings of patient biopsies.…”