Dr Valerie Callender on CCCA

In this podcast, Valerie Callender, MD, discusses the pathogenesis and treatment options for central centrifugal cicatricial alopecia in women with skin of color.

Dr Callender is a board-certified dermatologist in private practice in Glenn Dale, Maryland, and the past president of the Women Dermatologic Society and Skin of Color Society.

Transcript

Melissa: Hello. Welcome to another podcast with The Dermatologist. Today, I’ll be speaking with Dr. Valerie Callender about central centrifugal cicatricial alopecia. Dr. Callender is a board-certified dermatologist in private practice in Glenn Dale, Maryland, and is the medical director of Callender Dermatology and Cosmetic Center.

She is also the past president of Women’s Dermatologic Society and Skin of Color Society. Thank you so much for joining us today, Dr. Callender.

What is CCCA, and what is known about the pathogenesis?

Dr Valerie Callender: Central centrifugal cicatricial alopecia, and we always refer to it as CCCA, is a form of hair loss that is probably the number one cause of hair loss in women of color.

Basically, it’s a condition that presents with an inflammatory scarring, which is permanent form of alopecia. That inflammation causes total destruction of the actual hair follicles, so these patients have permanent hair loss.

As far as the cause of CCCA, like many of the inflammatory scarring alopecias, because this is just one of them, is multifactorial, and it’s also difficult to pinpoint what’s the ideology.

We do know that there is a genetic component. There’s a hereditary factor that’s been found in one South African study, that it’s autosomal dominant, so we know that there is a genetic component.

There’s also been recent developments looking at some of the genes that are associated with CCCA. One of them is a fibroproliferative gene. Authors, researchers at Hopkins actually identified that there’s an upregulation of a fibroproliferative gene, and it’s also associated with fibroids and leiomyomas.

There’s also a recent publication in the New England Journal of Medicine, that there is a mutation in PADI3 which is important in the hair shaft development. This has been identified in not only African American patients, but also in African patients, so we know that there is a genetic component to this condition.

We also know that although it’s genetic, there’s always missing pieces. It’s also associated with environmental factors. Most likely, there’s an association with hair grooming practices. There’s also a lot of research looking at some comorbidities such as endocrine disorders as well.

Melissa: What are the classical signs of CCCA, and how can dermatologists differentiate it from other alopecias?

Dr Callender: We do know that although CCCA is very common in women of African descent, we know that the most common type of hair loss in all women regardless of race is androgenetic alopecia, or female pattern hair loss.

Both these conditions, CCCA and female pattern hair loss occur centrally on the vertex of the scalp. Although you have some thinning at the top, the differential diagnosis includes CCCA in women of color but it also includes female pattern hair loss, particularly if they’re post-menopausal.

The way you tell the difference between the two, because they’re in the same location, definitely, we always start with history. CCCA, as I stated, is an inflammatory type of hair loss, and because it’s inflammatory, there are usually symptoms. Patients will complain of itching, tenderness, soreness on the top of their scalp.

Sometimes, they have no symptoms. Those are the hardest patients to treat, or those are the patients who have more extensive disease because they don’t know that they’re having issues, because they’re not having any symptoms.

You think about it, you shouldn’t know you have a scalp, right? You shouldn’t know when you have one, and if you know you have one, then usually that means there’s some kind of pathology going on.

In addition, you don’t know what your scalp really looks like. You rely on your hairstylist, you rely on your dermatologist, or a family member, or girlfriend to actually look at the top of your scalp and tell you what’s going on.

One of the ways to differentiate between CCCA and female pattern hair loss is, again, symptoms. Usually, patients with female pattern hair loss are asymptomatic.

You also have to think about the physical exam. When you examine the scalp, these are two different forms of alopecias. One, again, CCCA is an inflammatory scarring, and female pattern hair loss is a non-scarring form of hair loss.

Sometimes, you can actually see the difference. Scarred scalp looks a certain way. It feels a certain way. It doesn’t look healthy, and you can always compare the top of the scalp with occipital area in the back of the scalp, and you can see that there’s a difference.

If physical exam fails you, you can always use your dermatoscope, and you can look at the scalp and look at the hair follicles. There is a characteristic white-gray peripilar follicular halo that we see in patients with scarring alopecia or CCCA, which is pretty classic.

When you think about female pattern hair loss, you actually see the vellus hairs, because you get miniaturization of the hair follicle. You can tell the difference between the two just by using your dermatoscope. Again, if all else fails, skin or scalp biopsy is necessary, and you can definitely distinguish between the two.

You have to remember, you may have signs and symptoms of both. Physical exam, as well as dermatoscopic exam, as well as biopsy, patients may have both conditions, so there is an overlap in some cases.

Melissa: What therapeutic options are available for this condition?

Dr Callender: The treatment options that we have for CCCA is limited. The main goal of therapy is to stop the inflammation and stop the progression. You have to tell the patients that. There’s no way that you can take a permanent scarring type of alopecia and turn it around.

Your goal of therapy, number one, is to stop the spread, and you do that with anti-inflammatory agents. Of course we use doxycycline for its anti-inflammatory effects. We use topical corticosteroids. We use intralesional corticosteroids, and we also use anti-fungal shampoos that have an anti-inflammatory effect as well.

I think what’s important is to tell patients that there are two stages or phases of treatment. One is to decrease inflammation. The other one is the regrowth.

The first part is getting rid of that inflammation. Although you’re using oral medications, topicals and intralesional, you have to remember that it varies as far as how long it’s going to take for the inflammation to resolve.

I think one of the most effective treatments for this condition is intralesional cortisones, so, triamcinolone. What we usually do is, we use a dilution of 2.5 milligrams of triamcinolone acetonide, and we inject it directly into the scalp, usually about 1 to 2ccs, and sometimes 3ccs, based on the severity and the extent of involvement.

We inject it not just in the area of alopecia, but the focus needs to be at the periphery, in a way, like you’re just putting up a wall or just protecting those existing follicles from further destruction. Not only do we inject right in the center, but the most important is the periphery and going all around the area, trying to protect it.

We recommend a series of injections, and so, usually, patients come back every four to six weeks. You say, “Well, how many times?” I would say that most of the hair experts will say anywhere from five to eight treatment sessions.

Five is usually what we do, and then we begin the regrowth phase because, hopefully, the inflammation has subsided, and you can use your dermatoscope now to see if the inflammation has improved. Back in the days, we would do biopsy of the scalp to see not only the diagnosis at the very beginning, but also to see if your treatment was successful.

We recently published a study in the JDD using clobetasol, which is high potency topical cortisone in patients with CCCA. We actually biopsied the patients prior to treatment, treated them with clobetasol for an extended period of time, and then re-biopsied them at the end of the study. The symptoms improved.

The dermatopathogist, who was blinded on the biopsy specimens, showed a decrease in the inflammation after treatment with topical corticosteroids. This was the first paper that was published or study that actually looked at treatment, and why we do what we do.

We know topical steroids is anti-inflammatory. We’ve always known that, and that’s why we use it, but we’ve never had documentation or data to prove that it was very successful in this patient population.

When I think about the regrowth phase, that varies. A lot of us use topical minoxidil, which is off-label, but we use it in these patients. I think it’s a treatment option because, remember, these patients may also have AGA, or female pattern hair loss, so the addition of topical minoxidil in the later phase of treatment is recommended.

Melissa: Could you elaborate more on the specific cultural hair practices associated with CCCA?

Dr Callender: There’s a genetic component. It’s also hair grooming. I remember, back in the days, we use to call it hot comb alopecia, follicular degeneration syndrome, chemically-induced alopecia, and we just blamed it on hair grooming. We know now that that’s not true.

Do these hair care practices in hair grooming have a role, and is it an exacerbating factor in some patients? Absolutely. I think that a discussion always has to be top of the list and part of the exam or the visit in these patients, because they could be practicing hairstyles that are traumatic, and that may be irritating their scalp and causing more inflammation.

We do go through their hair care practices as part of their therapeutic management.

Melissa: In addition to discussing hair care, are there any other important considerations dermatologists should keep in mind when talking to patients?

Dr Callender: We always have to remember that we do know that this treatment, we have prescription medications. We know that they need to use these medication, but compliance tends to be an issue, and I’ll tell you, that goes on that office visit. It’s not a quick one.

It’s a visit that is very emotional to the women who suffer with CCCA, and so, we as clinicians, as dermatologists, need to be able to listen and then give good information.

As far as hair grooming, it’s very unique for women of color. Number one, we need to actually examine their hair and their scalp. That’s one thing I think is really important, is that you have to dive in to where they have their hair loss. [laughs] You can’t look at them from across the room and say, “You have alopecia.”

You have to get in there and part the hair, look at that scalp, feel the hair. You have to remember that, with African American hair, it tends to be very dry and brittle, and it’s because of the shape of the hair follicle or the hair shaft. It’s not coated adequately.

An individual who has very straight hair, the sebum oils just lubricate the entire hair shaft, whereas patients with black or African hair, it tends to be very dry and brittle because it’s not coated properly, and it intertwines, it curls around, there’s a lot of breakage, and so the hair is very fragile and it’s very delicate.

That’s one of the reasons why women of color straighten their hair. Whether they do it with chemicals or whether they do it with heat is an individual preference.

I will say that more women of color are keeping their hair natural. They’re not using these traumatic methods to straighten their hair. They’re just embracing the curly nature of their hair and just using really good hair moisturizers.

I think it’s important as dermatologists we understand that the hair is different. When it’s curly, the manageability is sometimes a problem, an issue, and so there are certain questions you need to ask your patient.

Number one, hair washing is important because that’s definitely different in women of color. Women of color, we actually wash our hair probably once a week or every two weeks, so that’s very important.

When you’re prescribing a shampoo, therapeutic shampoo for them, like the seb derm, or patients of psoriasis as well, you need to ask them the frequency of hair washing because you can’t tell them, “Take the shampoo, use it every day, and wash you hair under shower.” A woman of color is not going to do that.

If her hair is very short and she doesn’t have to worry about styling, she may be able to do that, but most women of color wash their hair once a week or every two weeks.

Some of them don’t do it themselves. I would say probably the majority don’t do it themselves, so they go to the salon. Sometimes, you have to tell them, “You’ve got to take the shampoo with you to the salon, let it sit on the scalp for about 15 minutes, rinse it out, and then the stylist can use their daily or their moisturizing shampoo and conditioner that they normally use.”

You have to incorporate your therapeutic shampoo into what they normally do. I think that’s very important.

I also think that, because we’re prescribing topical cortisones to their scalp, you have to ask them, as far as the vehicle, what their preference is.

Most women of color, because the hair is already innately dry and brittle, solutions tend to be very drying to the scalp and the hair, and can cause hair breakage. That’s a very important point because that’s a common complaint with women of color, that their hair is breaking.

Not only are they losing it from the scalp, they could also be compounding it with hair breakage, so it’s important to ask them about the vehicle.

We typically will ask them, what’s their preference? “Do you want something light, like a lotion or a foam? Do you prefer a more ointment-based, or an oil-based product?” Majority of patients of color are going to select an oil-based formulation or an ointment-based.

It’s important to ask them because, you think about compliance, if you’re going to give them something that’s going to break their hair off even more, they’re not going to use it, so, just ask them. Say, what do they prefer?

A lot of times, they’ll say, “Doctor, what do you recommend?” Then, you’ve got to know, “Well, OK, ointments tend to placate the scalp a little bit more, and you don’t have to use it every single day because you’re not washing it out in the shower every day, so you can apply the topical cortisone therapy maybe three times a week, and may not have to be every day.”

I think those are some of the important tips as far as hair care in women of color.

Melissa: What additional research is needed to improve treatment and the understanding of CCCA?

Dr Callender: I think we touched base a little bit about the ideology and the pathogenesis, since it’s unclear, and so I think a lot of the research, which is starting to come about, are definitely the other comorbidities, and whether we know it’s genetic now, we know that, which we didn’t know before. We assumed it, but we didn’t know it.

Are there any other comorbidities that put these patients at increased risk? Of course the literature has some small studies that actually look at the association with leiomyomas, association with some endocrine disorders. I think larger studies looking at some of these comorbidities is really important.

I also will say, as far as research, different treatment modalities, a lot of us now are using PRP or platelet-rich plasma to treat non-scarring alopecia or androgenetic alopecia, and whether these growth factors have anti-inflammatory capabilities to help patients with these inflammatory scarring alopecias.

I think there’s a lot of gaps, and hopefully, we’ll be able to offer more guidance when it comes to treatment as well as early recognition.

Melissa: What key takeaways would you like to leave with our audience?

Dr Callender: Some key takeaways that I would like to discuss and relay to the audience is basically just to be a compassionate clinician, and to know that hair loss in women, regardless whether the causes, is devastating. It definitely impacts the quality of life. It also affects their self-esteem, their confidence, their social interactions, their personal life.

You have to remember that, to be a good listener, to be compassionate, show empathy and understanding what they’re going through. I can’t tell you how many times I have a woman who suffers with hair loss to tell me that she went to a dermatologist or several prior, and that they told her, “Oh, there’s nothing we can do. Stop relaxing your hair. Wear a wig,” and send them on their way.

I think that is something that, as a physician, we should never do. Always give your patient hope and be very considerate of what they’re going through, and help them to understand what their condition is.

Give them a diagnosis. That’s another thing I hear. Patients will say, “I have hair loss. I have alopecia,” and you ask them, “Well, do you know which form or the type that you have?” and they’re like, “No, the doctor just told me I have alopecia.”

I think patients deserve to know what they have, and we have to take those extra steps and do the studies, biopsy, dermatoscopic exam, etc., and come up with a diagnosis and a management plan for them.

I think they also need to know that there’s no cure. Not only are you going to treat them with active therapy, but then there’s maintenance involved. You don’t know the cause of it, so there’s no cure. It may start destroying follicles the following week, so you can’t really stop therapy. You’ve got to keep an eye on your patients.

I will say, in closing, that hair loss is definitely devastating to women, and we have to take it serious.

Melissa: Thank you again for joining us today, Dr. Callender. If you have any questions for Dr. Callender, or enjoyed this podcast, please submit your comment or question in the feedback box below. We appreciate your feedback, and thank you for listening.