Updates in Central Centrifugal Cicatricial Alopecia

Crystal Aguh, MD, discussed the unique clinical and histologic presentations of central centrifugal cicatricial alopecia (CCCA) and identified standard and emerging treatment options in her session, “Updates in Our Understanding of CCCA,” during Dermatology Week 2023.

Dr Aguh is an associate professor in the department of dermatology and director of the ethnic skin program at Johns Hopkins School of Medicine in Baltimore, MD.  

CCCA is the most common cicatricial alopecia in Black women. Typically, hair loss begins at the vertex and progresses outward in a centrifugal pattern. The link to hairstyling practices is unclear and likely overemphasized.

In a significant proportion of women with CCCA, recent data have identified a mutation in the PADI3 gene, which is responsible for hair shaft formation. Data also suggest that CCCA may be inherited and overlap with other diseases of abnormal scarring. One theory is that genetically susceptible individuals have an exaggerated response to an inflammatory trigger, which leads to an increase in the TGF-ß, or scarring, pathway, with the counter-regulatory PPARγ pathway insufficient to halt the fibrotic cascade.

Dermoscopic findings demonstrate a peripilar gray-white halo corresponding to follicles missing the inner root sheath. CCCA can insidiously progress to end-stage fibrosis that is disproportionate to clinical signs of inflammation. Histologic identification of CCCA can be difficult because it shares overlapping features with other cicatricial alopecias, especially those in later stages. A hallmark histopathologic finding may be premature desquamation of the inner root sheath in noninflamed follicles.

Erythema and pruritus may be present. Because pruritus is associated with active inflammation, it is important to monitor for itching in patients with CCCA. Regarding other clinical monitoring, patients with CCCA are more likely to have a history of type 2 diabetes compared with age, race, and sex matched control individuals, according to a recent study.

Standard therapy for CCCA includes topical and intralesional steroids to slow its progression. Tetracycline derivatives may be helpful for patients experiencing more profound inflammation.

Dr Aguh has added topical metformin 10% to 20% compounded in lipoderm cream or clobetasol ointment to standard therapy for patients who have undergone multiple rounds of intralesional Kenalog and topical steroids with limited improvement. When applied to the scalp once daily, this has shown disease improvement, including some patients achieving full scalp coverage.

She noted, “I typically like this for premenopausal patients who do not have a lot of androgenic alopecia, they have true scarring and there are very few options for them. If I have a postmenopausal patient with CCCA, one of the first things I am going to do is add topical compounded minoxidil or oral minoxidil to really take advantage of that androgenic alopecia component.”

“Overall, the future is very bright for these patients,” Dr Aguh concluded.

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Reference

Aguh C. Updates in our understanding of CCCA. Presented at: Dermatology Week; May 3–6; Virtual.