What is Central Centrifugal Cicatricial Alopecia (CCA)?

Central Centrifugal Cicatricial Alopecia (CCCA) belongs to a group of disorders called cicatricial or scarring alopecias that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. The cause is unknown but is probably an inflammatory process induced by genetic or environmental factors. Women with CCCA develop an area of thinning at the center of their scalp, and this gradually enlarges and spreads outward, hence the name “central centrifugal”. The inflammation that destroys the hair follicle is below the skin surface and there is usually no “scar” seen on the scalp.

Cause

The cause of CCCA is unknown. Genetic factors may be important, and inflammation plays a role in the process. The inflammation, in the form of white blood cells, surrounds the hair follicles and gradually destroys them.

Signs & Symptoms

CCCA affects primarily women of African descent, but rarely occurs in women of other ethnic groups and men. It usually presents in individuals 25-65 years of age. There have been several reports of CCCA occurring in families and genetic studies are currently underway to further identify possible contributing genes. However, most patients with CCCA have no family history of a similar condition.  CCCA is not associated with any other illnesses.

In many cases, the area of hair loss of CCCA is not accompanied by any symptoms. In some cases, the hair loss is associated with itching, burning, tenderness, pain or a tingling sensation. The bare area gradually enlarges and may affect a large portion of the central scalp with only few solitary hairs remaining. Two other hair problems, hair breakage and traction alopecia (hair loss along the hairline), may be present in some women with CCCA, but it is not clear if they are at all related to the scarring process. Hair breakage and traction alopecia are both due to hair styling and hair grooming practices and the relationship to the development of CCCA is still being determined.

Diagnosis & Treatments

Experienced dermatologists will suspect the diagnosis of CCCA from a detailed clinical history, thorough physical examination of the scalp, and scalp biopsy.  A scalp biopsy the gold standard of diagnosis of scarring hair loss and are often performed when considering a diagnosis of CCCA.  The biopsy procedure is performed in the office with a local anesthetic, and a small skin sample is taken for microscopic examination. The most helpful information from the biopsy is the extent of inflammation, number of hair follicles present, and the amount of scar tissue, which are all used to select appropriate therapy.

Early diagnosis and therapeutic intervention can often prevent permanent damage to hair follicles. Once a definitive diagnosis is made, we can determine the best treatment plan for each individual. Some regrowth is even possible with treatment.

The goals of treatment are to relieve symptoms, signs, and halt spread of the disease. Hair regrowth is not possible after the hair follicles are replaced by scar tissue. For this reason, it is important to start treatment early before the hair loss is extensive.

Treatments include anti-inflammatory medications to decrease the inflammation surrounding the hair follicle and prevent its permanent destruction.  In mild cases, topical corticosteroids in the form of ointments, oils, solutions, lotions, foams, sprays or shampoos can help decrease inflammation and calm associated symptoms (itching, redness, or pain).  Topical tacrolimus may also be helpful as a steroid sparing topical agent.  Additionally, injections of corticosteroid, such as triamcinolone acetonide, may be used in inflamed and symptomatic areas, every 4-6 weeks. In more advanced cases, the addition of oral medications is needed such as tetracycline antibiotics (used for its anti-inflammatory properties), or hydroxychloroquine (an antimalarial drug with anti-inflammatory properties). Topical minoxidil may be useful in the form of solution or foam to keep the remaining follicles in their growing phase longer and stimulate longer hair growth. Hair transplantation is an option only in selected cases and after the inflammation has been controlled and hair loss is no longer spreading.

Women with CCCA should handle their hair gently and may continue to use their usual shampoo and conditioner if there is no breakage or traction alopecia. When hair breakage is present, the hair is short and seems to have “stopped growing”. It is then important to avoid excessive heat and chemical processing.  Perms and relaxers should also be minimized, and the interval between them increased to 8 weeks or longer. A hair piece or wig is a good way to give hair a rest from heat and chemicals. When traction alopecia is present, avoid pulling hair styles such as tight ponytails; braids and corn rows should be loose and not pulled tightly at the scalp; heavy hair extensions should be avoided.

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