Central Centrifugal Cicatricial Alopecia

Central centrifugal cicatricial alopecia (CCCA) is a lymphocyte-predominant scarring alopecia characterized by permanent hair loss that classically begins on the crown of the scalp and gradually spreads centrifugally. It predominantly affects women of African descent, with estimates suggesting a prevalence of up to 7%. However, no population-based studies have been performed, so its true prevalence remains unknown.

CCCA is believed to result from a complex interplay of genetic susceptibility and environmental factors, though theetiopathogenesis is poorly understood. The historically posited role of haircare practices is uncertain, though superimposed acute (e.g., chemical, thermal) or chronic (e.g., traction) insults may play a role. Genetic determinants have begun to be elucidated, most notably with the association of variants in peptidyl arginine deiminase type III (PADI3) and pleiotropic effects on other follicular proteins.

Clinically, CCCA often presents with progressive scarring alopecia involving the crown, vertex, or nearby scalp that expands outward over time. Patients may report pruritus, burning, tenderness,or scalp dysesthesia, although many remain asymptomatic and first notice localized hair breakage or thinning. Examination may reveal loss of follicular ostia, perifollicular scale, follicular papules or pustules, andareas of scarring alopecia. In CCCA patients with richly pigmented skin, perifollicular hyperpigmentation may also be appreciated. Histopathologic examination demonstratesa CD4-predominant lymphocytic infiltrate with premature desquamation of the inner root sheath, perifollicular fibroplasia, and progressive fibrosis, ultimately resulting in permanent destruction of the pilosebaceous unit. Trichoscopy is a valuable diagnostic adjunct and commonly demonstratesabsent follicular openings, white peripilar halos, perifollicular scale, and perifollicularhyperpigmentation. Although not specific, a soft scalp may also be appreciated on palpation in some CCCA patients.

Hair regrowth is not possible oncefollicles have been permanently scarred, so treatment focuses on reducing inflammation and preventing disease progression. First-line therapy includes high-potency topical corticosteroidsand intralesional triamcinolone acetonide. Since corticosteroids may produce hypopigmentation in patients with Fitzpatrick IV–VI, the lowesteffective dose should be used whenever possible. Other options include oral tetracyclines, particularly doxycycline, which are frequently used for their anti- inflammatory effects and are typically continued for two to six months. Patients with progressive or refractory disease may require systemic immunomodulatory therapy, including hydroxychloroquineor mycophenolate mofetil. Hair transplantation or other surgical restoration procedures may be considered in carefully selected patients whose disease has remained clinically inactive for at least nine to 12 months. Of note, emerging therapies such as topical 10% metformin, oral Janus kinase (JAK) inhibitors,and apremilast (Otezla, Amgen) have demonstrated encouraging outcomes in preliminary CCCA case series and pilot studies. However, robust randomized controlled trials are required before their routine use can be recommended.

Beyond permanent hair loss, CCCA has a substantial psychosocial impact. Studies of women with scarring alopecia have demonstrated significant impairment in quality of life, with embarrassment and bothersome physical symptoms representing the two greatest contributors. Maintaining a strong patient–clinician relationship is ideal. Additionally, patients should be counseled on gentle haircare practices. Hairstyles that produce prolonged tension, excessive heat, or repetitive mechanical trauma should be minimized, if possible, and frequent use of chemical relaxers is discouraged because these practices may contribute to disease progression and ongoing follicular injury.

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