Central Centrifugal Cicatricial Alopecia - Causes, Clinical Features, and Treatment

Introduction:

Central centrifugal cicatricial alopecia is a type of alopecia that is characterized by permanent loss of hair. This condition, although in rare cases, can be seen in men and women of all races. It is most commonly seen in African and Middle-aged women. The causes and treatments are multifactorial. In addition, the treatment involves an interprofessional approach.

What Is Central Centrifugal Cicatricial Alopecia?

Central centrifugal cicatricial alopecia is a condition in which hair loss is noticed on the crown of the scalp. The hair loss then progresses centrifugally. It is also associated with signs of inflammation and is commonly seen in women of African descent. It also has a strong familial association. This type of hair loss is also associated with inflammatory signs and symptoms.

What Is Alopecia?

To properly understand CCCA, one has to first understand alopecia. Alopecia areata arises when the immune system attacks the hair follicles, causing hair loss. It is a common autoimmune disorder that results in unpredictable loss of hair. It can affect individuals of any race, gender, or age. There is, unfortunately, no cure for alopecia, but standard treatment options include corticosteroids and anti-inflammatory agents which target the immune system. Generally, alopecia develops during childhood or during the teenage years. Depending on the extent of the area where hair is lost, alopecia can be divided into:

• Alopecia Areata: Hair loss occurs in patches.

• Alopecia Totalis: Entire hair on the scalp is lost.

• Alopecia Universalis: The person loses all body hair.

What Causes Central Centrifugal Cicatricial Alopecia?

• There are several different factors that can lead to the condition, and therefore it is a condition that requires the role of an interprofessional team to manage it. However, the exact cause of CCCA remains unknown. It has been suggested that a genetic mutation of the gene PAD13 could be responsible for the disease. The gene PAD13 encodes peptidyl arginine deiminase, type III, which is the enzyme that is essential for the formation of hair shafts.

• Other likely factors include practices such as the use of a hot comb, relaxers, weaves, and tight extensions. However, a direct correlation between them has not been established. The presence of the condition in individuals who do not follow such practices has weakened this suggestion.

• Factors such as bacterial and fungal infections and autoimmune disorders are the other factors proposed.

• Medical conditions have also been observed to have an association with the disease for instance type 2 diabetes mellitus.

What Are the Clinical Features of Central Centrifugal Cicatricial Alopecia?

• The typical feature of central centrifugal cicatricial alopecia is, like the name suggests, hair loss at the vertex or middle of the scalp. This hair loss extends outwards in a centrifugal fashion.

• On examination, it can be observed that there is a loss of follicular openings, and this causes the scalp to appear shiny.

• In some people, symptoms such as itching, tenderness, and burning sensation may be present.

• An early sign of CCCA is hair breakage, followed by a slowly progressive hair loss.

How to Diagnose Central Centrifugal Cicatricial Alopecia?

• It is crucial to diagnose the condition at an early stage, as medical intervention can prevent the worsening, which results in extensive and permanent loss of hair.

• Diagnosis can be based on the clinical features and elimination of other hair loss disorders.

• A scalp biopsy can be performed for confirmation of the condition. Sample for scalp biopsy should be taken from an active edge of alopecia instead of the center of a scarred area. On examination, inflammatory cells around the base of the hair follicle and fibrosis can be observed. Premature peeling off (desquamation) of the inner root sheath is another common feature observed.

How to Treat Central Centrifugal Cicatricial Alopecia?

1) The primary goal is to intervene in the progression or to worsen the condition, thereby preventing further hair loss.

2) In the areas where fibrosis has already occurred, regrowth is not possible. And because the underlying cause remains unknown, there is no targeted therapy available.

3) Since a number of factors are considered to be the cause of the condition, different approaches can be undertaken for the treatment, including medications, surgical or cosmetic procedures, and gentle hair care practices.

4) A few treatment options that have been suggested include anti-inflammatory agents. This is because inflammatory agents have been found in the base of the hair shaft, suggesting that inflammation may have a role to play in hair loss. Such agents are:

• Topical steroids such as Clobetasol or intralesional steroids.

• Tetracyclines: Doxycycline 100 mg twice daily can be taken for several weeks to months.

• Calcineurin Inhibitors: Pimecrolimus cream or Tacrolimus cream.

• Cyclosporin.

• Hydroxychloroquine.

• Minoxidil solution has been recommended to help stimulate hair growth in follicles that are still viable.

• Seborrhoeic dermatitis, if present, should be treated with appropriate medicated shampoos and topical anti-inflammatory agents.

The other treatment options are listed below:

1. Hair transplant is another option, but it is suggested only for individuals with well-controlled CCCA for at least a year. The limitation of hair transplant is the low graft survival rate.

2. Women are encouraged to consider natural hairstyles and discontinue traumatic hair care practices.

3. Relaxers, if performed, should be done by a professional, with a gap of six to eight weeks, and care should be taken to make sure the scalp is not burned during the process of relaxer application.

4. Avoid tight braids, extensions, or weaves.

5. Minimize the use of heat applications such as blow dryers, hooded dryers, hot combs, and flat irons.

6. Avoid hairstyles that can cause scalp irritation, discomfort, or scale.

7. Shampooing and the frequency of it varies from individual to individual. Most use shampoo every one to two weeks. This prevents excessive dryness.

Conclusion:

Much like the name suggests, central centrifugal cicatricial alopecia is a type of hair loss that is noticed on the crown or vertex of the scalp and proceeds centrifugally. Typically it affects middle-aged women of African ethnicity. However, it is not exclusive and can affect men and women of all races. Treatment involves an intervention to prevent further hair loss, practices to reduce stress on hair follicles, and medications that are anti-inflammatory.