Dermatological Conditions in SKIN OF COLOR— A Practical Approach to Centrifugal Cicatricial Alopecia

Centrifugal cicatricial alopecia (CCCA) is the most common cause of scarring alopecia in African American women. As the name indicates, it is hair loss that begins at the vertex or midscalp and spreads centrifugally.1

Several studies have reported varying prevalence rates of CCCA in the United States (US). One study with 529 African American women reported a prevalence rate of 5.6 percent.2 Another study with 326 African American women reported a prevalence rate of approximately 28 percent.3 Both of these studies were conducted in 2011. There is need for more recent and larger scale studies to determine the true prevalence of CCCA.

When the condition was first described in 1968 and named “hot comb alopecia,” it was thought to be due to common haircare practices of African American women. Indeed, some haircare practices, such as traction styles, cause an inflammatory reaction of the scalp and lead to scarring.4 However, recent research has shown that, while hair care practices may contribute to the development of CCCA, they are not the cause. CCCA is thought to be multifactorial, with genetics also playing a role. For example, a study by Dlova et al5 looked at 14 Black South African families and found there to be an autosomal dominance inheritance pattern with partial penetrance. There was a positive correlation between traction styles and severity of CCCA. However, six patients with CCCA did not have a history of traction styling.5 This demonstrates that while haircare practices may contribute to the development and progression of CCCA, they should not be assumed to be the sole culprit.

As with many dermatological diseases, there is more to CCCA than meets the eye. Recently, a small study of five patients by Aguh et al6 found that those with CCCA have an upregulation in genes that are found in other fibroproliferative disorders (i.e., keloids). New research has also demonstrated a possible association between CCCA and several systemic diseases, such as Type 2 diabetes mellitus3 and uterine leiomyomas.7 More research is needed to understand the implications of these findings.

PATIENT HISTORY

A comprehensive patient history is important for diagnosis. See Box 14,8 for a list of questions that are helpful in aiding a diagnosis of CCCA.

CLINICAL PRESENTATION

Early diagnosis and prompt treatment of CCCA leads to the best possible treatment outcome. Early in the disease, the crown of the scalp has subtle hair thinning. As the disease progresses, central scarring begins to occur with an increase in hair thinning. In late stages of CCCA, there is widespread hair loss with scarring. In addition to hair thinning and loss, patients may also experience scalp symptoms, such as burning, pruritis, erythema, tenderness, flaking/scaling, and/or pustules.9

BOX 1. PATIENT HISTORY QUESTIONS4,8.

• What was the age of onset?

• What is the disease duration?

• Are existing lesions stable or worsening?

• Are there new lesions?

• Has the disease been previously treated? If so, how and when?

• Is it being actively treated? If so, how?

• Is there burning, itching, or tenderness? If so, how often and how severe on a scale of 1–10 (with 10 being the worst)?

• What is the patient’s gynecological history and menopause status?

• What is the patient’s haircare history (i.e. use of relaxers, braids, etc.)?

• Is there a family history of the disease?

DIFFERENTIAL DIAGNOSIS

Early in its presentation, CCCA can often be mistaken for other alopecic disorders, the most common of which are traction alopecia, androgenetic alopecia, discoid lupus erythematosus, and lichen planopilaris (Table 1).10–15

TABLE 1.

List of differential diagnoses

DISEASE	KEY CLINICAL FINDINGS
Traction alopecia10	“Fringe sign:” hair follicles of lesser diameter are retained along the frontal/temporal hairline History of hair practices that involve pulling (e.g., braids, cornrows, twists, ponytails, etc.)
Androgenetic alopecia11	Gradual thinning of hair at vertex of scalp Frontal hairline usually not affected Women often first complain of a “widening of their part” Ask about hyperandrogenism (e.g., polycystic ovary syndrome, hyperprolactinemia, etc.)
Discoid lupus erythematosus (DLE)12,13	Follicular keratotic plugging within alopecic patch Well-circumscribed, atrophic, white, smooth plaque with an elevated, active border Ask about personal and family history of DLE
Lichen planopilaris14,15	Multifocal or central hair loss with perifollicular erythema and scaling Hair follicles surrounding alopecic patch with perifollicular erythema and scaling Presence of itching/burning/pain/tenderness Look for nail and mucous involvement Ask about past history regarding lichen planus

DIAGNOSIS

A 4mm punch biopsy from an active area of inflammation is often performed to confirm the diagnosis. If in doubt, the border of an alopecic patch is a good place to biopsy. The sample needs to include subcutaneous fat, as this is where the anagen follicles are found. Common dermoscopic findings include perifollicular gray-white halo, loss of follicular ostia, and one or two hairs emerging together.8

TREATMENT

The goals of treatment are to halt disease progression and establish hair regrowth. The first step in treatment is to minimize inflammation. Recall that chronic and persistent inflammation leads to hair follicle destruction and subsequent scarring. Treatment during this inflammatory stage is multipronged and often involves a combination of high-potency topical corticosteroids daily or every other day, intralesional triamcinolone (TAC) 5 to 10mg/mL every 4 to 6 weeks for 3 to 6 months, doxycycline 100mg twice daily for three months, and an antiseborrheic shampoo every 1 to 2 weeks.16

The next stage in treatment is to encourage hair regrowth. Topical minoxidil daily (2% or 5%) is incorporated into the regimen to support hair growth. A maintenance regimen for patients often includes a midpotency topical corticosteroid three nights per week, daily topical minoxidil, an antiseborrheic shampoo weekly, with intralesional TAC as needed.16

In addition to medical treatments, recently, literature has been published on the positive impact of cosmetic procedures, such as platelet-rich-plasma (PRP) in the treatment of CCCA.17 More research needs to be conducted before PRP can be widely recommended.

PEARLS

1. See patients early and often. It is prudent during the first six months of diagnosis to see patients monthly for TAC injections and follow-up. After that, you can decrease follow-ups to every 2 to 3 months. Once patients are seeing significant regrowth and are comfortable with the treatment maintenance regimen, you can decrease visit frequency as appropriate.

2. When examining the patient, actually touch their scalp. This might seem obvious, but you would be surprised at how many patients cried in my exam room because I not only looked at their scalp, but touched it. This helps you become a more astute diagnostician and at the same time builds rapport with the patient.

3. Seek cultural competency training. There is widespread belief among patients that if a provider does not share the same racial or ethnic background as them, they are ill-equipped to treat them. Unfortunately, this belief often becomes a self-fulling prophecy for the patient when they see a provider who does not know how to care for their hair. Take the time to learn about cultural practices across races and ethnicities. Peer-reviewed journals and continuing medical education (CME) conferences are a great place to start.