Overview:

Central Centrifugal Cicatricial Alopecia (CCCA) is a form of scarring hair loss (alopecia) most common in African American women. There is a genetic basis for CCCA meaning it can run in families, however, hair care and styling practices are also linked to the condition. Patients experience progressive hair loss at the vertex of the scalp. Medication and hair care changes can slow progression, however, areas that have scarred do not generally regrow. In this article we will discuss causes, symptoms, diagnosis, treatment, and prognosis.

Causes:

The exact cause of CCCA is unknown, but it's thought to be a combination of genetic and environmental factors. Family history is a risk factor, as ccca can run in families. A recent study published in the New England Journal of Medicine showed PADI3 mutations are linked to development of CCCA (https://www.nejm.org/doi/full/10.1056/NEJMoa1816614). In addition, hairstyling practices that pull on the hair (such as tight braids or cornrows) are thought to play a role. CCCA is seen most often in African American women, which suggests that genetics may play a role. Although the exact number is unknown, it is estimated that CCCA may affect up to 15% of black women in the general population.

Symptoms:

The main symptom of CCCA is progressive hair loss at the vertex (top) of the scalp. The hair loss may start as thinning, but can progress to balding. In some cases, CCCA can also cause itching, burning, or scaling of the scalp.

Diagnosis:

CCCA is diagnosed based on a physical examination and medical history. Your doctor will ask about your symptoms and any family history of hair loss. Clinical presentation and dermoscopy is typically sufficient for a diagnosis, however, in causes of diagnostic uncertainty a scalp biopsy may be performed.

Treatment:

There is no cure for CCCA, but treatments can help slow the progression of hair loss. These include:

-Gentle hair grooming practices such as natural (chemical and heat-free) hair styles, reduced frequency of relaxers, and limited styling products, are also recommended in order to prevent inflammation and concomitant hair breakage. Reference: (https://skinofcolorsociety.org/patient-dermatology-education/central-centrifugal-cicatricial-alopecia-ccca/)

- Minoxidil:  Available as a prescription pill or as over-the-counter medication (Rogaine) applied to the scalp to promote hair growth. It must be used indefinitely to maintain results.

- Steroid injections: These are injected into the scalp to help reduce inflammation and promote hair growth.

-Topical steroids: Anti-inflammatory topical medications applied to the scalp to reduce inflammation and help promote hair growth. They are typically not as effective as steroid injections but are painless and can be applied at home.

- Oral steroids: These are taken by mouth to help reduce inflammation. They can have serious side effects and should only be used for a short time.

- Non steroid immunosuppressives: These are taken by mouth as steroid sparing long term options to limit progression. These often care some side effects including increased risk for infection.

-Hair transplantation: hair transplantation can be performed in CCCA, however, hair loss must be stable / quiescent for at least 1 year prior to proceeding. If your hair transplant surgeon is not also a dermatologist, you should be sure you are seeing a dermatologist as well to monitor the activity level of your CCCA. Any hairs transplanted into active CCCA will likely be lost with time.

Prognosis:

The prognosis for CCCA is variable. Some people who seek treatment early experience only mild hair loss, while others who delay treatment may lose all of the hair on their scalp. In general, CCCA progresses slowly and can be controlled with medication and lifestyle changes. However, scarring alopecia is permanent, meaning that areas that have scarred will not regrow.