Approach to Diagnosis and Treatment of CCCA

In this interview, Dr Loren Krueger discusses her session, “Central Centrifugal Cicatricial Alopecia (CCCA): Advances in Diagnosis and Treatment,” at the 2023 AAD Annual Meeting.

Dr Krueger is a medical and procedural dermatologist at Emory University in Atlanta, GA. Her areas of expertise include pigmentary disorders, hair loss disorders, and skin of color dermatology. She trains learners and residents through her role as associate program director for the dermatology residency. She also serves as the vice chair for diversity, equity, and inclusion for the department of dermatology.

The Dermatologist: What are the latest advances in CCCA?

Dr Krueger: CCCA has had so many updates in our understanding of the pathophysiology and the etiology. We know that this was originally coined hot comb alopecia, but the more we learn about it, the more we understand this is probably less likely associated with just hairstyling practices and has a strong genetic predisposition. Because it was noted that this was found in patients as young as age 11, it tends to be traveling in families, and is also reported in those who are using mainly natural styles, there was an increased curiosity about the role of genetics in this condition.

A couple of studies have come out regarding this, including a pedigree analysis of over 30 relatives from South Africa, which did find a proposed autosomal dominant inheritance. I do think there's more to be unveiled there, but definitely strong family trends in terms of CCCA. And that makes sense because The New England Journal of Medicine published a huge sentinel study looking at variants in PADI3, a protein responsible for hair shaft formation, which was found to be mutated in patients with CCCA. That's extremely important and helps us know there is this genetic association with CCCA. So, the more we think about it, we're thinking that there's some genetic predisposition that makes a person more likely to have fibroproliferation, which is clued in with the association with uterine fibroids, for example. And with some triggering environmental factor, less likely a role for just hot combs and relaxers, that's what leads to CCCA.

The Dermatologist: Can you discuss your treatment approaches to CCCA?

Dr Krueger: CCCA is really challenging. We know this is a cicatricial or scarring alopecia, so I like to lead with managing expectations from the beginning. Goals for treatment are really to stop progression. Maybe we can get back 10% to 20% of hair. I always lead with that. Of course, if I'm able to get back more, I would rather underpromise and overdeliver.

We can use a number of different things. I use topical steroids, usually ultrapotent clobetasol in combination with topical tacrolimus to the scalp, and topical minoxidil, acknowledging that topical minoxidil in foam or solution form can be drying to curly-textured hair. Thinking about getting that compounded in an ointment is another consideration. I do use intralesional injections. I've seen those described anywhere from 3.33 to 10 mgs/ml in CCCA, and orals, such as oral doxycycline or hydroxychloroquine. I will also use therapies to regrow hair, such as low-dose oral minoxidil. Recently, there have been a couple of cases of topical metformin, which we know improves fibrosis in a mouse model, that has been used at 10% compounded for recalcitrant cases of CCCA.

The Dermatologist: Can you go over the key points from your session?

Dr Krueger: Key points for my session are just to really think of this more as a genetic predisposition to fibroproliferation that leads to hair loss at the center of the scalp, de-emphasizing hairstyling practices to avoid placing guilt on patients. Of the 3 studies that have really emphasized hairstyling, no association has been found with hot combs or chemical relaxers, and instead there has been association with high-tension styling at the center of the scalp. So, if we are going to counsel about hairstyling, we do have to know the literature, that it's less likely implicated right now in the literature to be hot combs and chemical relaxers and more so high-tension styling, which we know can be dangerous for things like acquired trichorrhexis nodosa, as well as traction alopecia. I do emphasize there's probably a genetic predisposition to this. We're not really sure how hairstyling is playing a role. Caution with high-tension styles. Let's talk about a hairstyling regimen that works for the patient. And those intimate conversations and shared decision-making tend to go well in terms of developing a treatment plan that involves hairstyling practices for patients.

The Dermatologist: What future research is needed to understand the diagnosis and treatment of CCCA?

Dr Krueger: That's an excellent question. There were some key studies that came out of Dr Crystal Aguh's work in terms of fibroproliferation, looking at which proteins were upregulated. In affected areas of the scalp, there tends to be an elevation in matrix metallic proteinases, as well as platelet-derived growth factor, and some collagens as well. Overall, I think a better understanding of the molecular basis of what's going on is needed. Also, larger studies on the role of hairstyling, investigating things such as the various styles, braiding, high-tension ponytails, etc., vs chemical relaxers and hot comb use or heat use. I think we need to flush that out to either put an end to that or to really investigate how we can best counsel our patients. Those are some areas that I see really being ripe for further exploration in CCCA.

The Dermatologist: Is there anything else you would like to share with your colleagues about treating CCCA?

Dr Krueger: This is the most common type of scarring alopecia in Black women. And it is so devastating and has such an impact on quality of life, especially for those more severe cases. I think early recognition is key. Signs such as not necessarily perceptible loss at the center of the scalp, but also itching or breakage at the crown, bogginess, and those subtle dermoscopic findings that can be found early on like perifollicular halos are going to be key in making this diagnosis early so we can have patients hold onto the hair that they do have and maintain comfortable styling.

Reference
Krueger L. CCCA: advances in diagnosis and treatment. Presented at: AAD Annual Meeting; March 17–21, 2023; New Orleans, LA.