Atypical CCCA Hair Loss Remains Underappreciated

New York — The diagnosis of central centrifugal cicatricial alopecia (CCCA), the most common form of cicatrizing hair loss in Black women, is still being missed, resulting in delayed diagnoses and inappropriate treatment because of various factors that include atypical presentations.

CCCA, which typically features the key descriptive characteristics captured in its name, is easier to miss than is widely appreciated, according to Susan C. Taylor, MD, professor and vice chair of diversity, equity, and inclusion in the Department of Dermatology, University of Pennsylvania, Philadelphia.

In a recently published scoping review of 281 CCCA cases described in 99 studies, Taylor and co-investigators found that about 70% patients had the classic appearance of hair loss in the central portion of the scalp expanding in centrifugal fashion, but she cautioned that nearly one third did not.

Atypical CCCA Presents in Two Major Types

“There were two distinct types of atypical presentations,” she said in a presentation at the 2024 Skin of Color Update. In both, loss of hair at the vertex of the skull was not a prominent feature.

In one presentation, hair loss was best characterized as patchy and could involve the occipital, parietal, or frontal areas of the skull. In the other, hair loss most closely resembled androgenetic alopecia or, in one case Taylor presented, secondary syphilis. Trichoclasis was identified in a large proportion of patients, and parietal involvement was common.

Cautioning clinicians to be aware of the limitations of the classic CCCA nomenclature, Taylor said that failure to consider CCCA in Black women presenting with hair loss can lead to a misdiagnosis, a delayed diagnosis, and inadequate therapy.

“CCCA is a progressive disease, so we do not want to miss or delay the diagnosis. We want to start therapy as quickly as possible,” she said.

She said that the same principles apply to Black men, whose risk for CCCA is underappreciated. Even for those who consider CCCA in men, it is important to consider atypical presentations, according to Taylor, an author of a recently published review of all CCCA cases among men in a University of Pennsylvania database.

“Here is the bottom line. If you are thinking of androgenetic alopecia in a male patient of African descent, you have to think of CCCA,” she said.

Dermatoscopic Diagnosis Useful in Men and Women

In both men and women, suspicion of CCCA should lead to a dermatoscopic examination and a scalp biopsy. Key dermatoscopic features include a honeycomb pigmented network and a peripilar white halos. These have a diameter of 0.3-0.5 mm around the hair follicle and may be accompanied by pinpoint white dots that are about the same size. White macules, which present in a “starry sky pattern,” also provide dermatoscopic support for a diagnosis of CCCA.

The use of objective tests, such as dermatoscopy or trichoscopy, was also strongly encouraged by Amy McMichael, MD, professor of dermatology at Wake Forest University, Winston-Salem, North Carolina. She, too, spoke about CCCA at the 2024 Skin of Color Update and reinforced the importance of considering atypical CCCA.

“We need to think more globally,” McMichael said, encouraging examination of the posterior scalp as soon as CCCA is considered. Rather than just prevent further hair loss, McMichael said her goal is to rescue hair follicles so they can be revived to function normally after the inflammation of CCCA is controlled.

Once thought to be the consequence of chemical relaxers and other products used for hair styling, CCCA is now considered often to be the result of a genetic predisposition, according to McMichael. Indeed, while she counsels patients with CCCA to discuss genetic risk with other relatives, she no longer advises all patients to immediately abandon chemical relaxers.

Causative Role of Hair Products Unclear

Noting inconsistencies in the evidence that have linked CCCA risk to the use of hair products, McMichael does not focus on this as a key etiologic factor. Rather, she places a great emphasis on therapies that will rapidly control inflammation and considers the control of inflammation to be a prerequisite for achieving durable remissions.

When evaluating the scalp, she highly recommends assessing the degree and severity of inflammation as a first step toward a treatment plan. “For patients in the inflammatory stage, I really try to hit hard,” she said. Although she individualizes treatment in regard to the patient’s ability to tolerate aggressive therapy, she considers topical and intralesional steroids, as well as oral antibiotics for treating pustular disease.

Once inflammation is controlled, she educates patients about the variety of options that can be considered to maintain control, such as topical minoxidil or, in the event of difficult cases, plasma-rich protein (PRP), laser treatment, or surgery. In all cases, these approaches should be introduced after the inflammation is controlled.

However, CCCA must be recognized as a chronic disorder, McMichael said. She warned that topical treatments and PRP are best recognized as maintenance interventions. Patients often stop treatment once they see hair regrowth, but this is a mistake, she said. Even if the reasoning is that the treatment is costly or inconvenient or that their CCCA is cured, patients must be dissuaded.

“If you start patients down this road, they are going to have to stay on their management, or they are going to lose hair again,” McMichael cautioned.

Taylor, president-elect of the American Academy of Dermatology, reported financial relationships with more than 25 pharmaceutical and cosmetic companies. McMichael reported financial relationships with AbbVie, Almirall, Arcutis, Bristol Myers Squibb, CeraVe, Eli Lilly, Galderma, Janssen, Johnson & Johnson, Leo Pharma, L’Oréal, Pelage, Pfizer, Procter & Gamble, Revian, Sanofi Regeneron, Sun Pharma, and UCB.

Ted Bosworth is a medical journalist based in New York City.