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Characteristics and Affected Patients
Central centrifugal cicatricial alopecia (CCCA) is a disease characterized by permanent hair loss in the crown region of the scalp, inflammation, and scarring. It occurs almost exclusively in black women aged 30 to 55 years. It is estimated that CCCA may affect up to 15% of black women, but the precise number of individuals affected in the general population is unknown.
There are likely multiple causes of CCCA. Recently, a gene variant was discovered in about 25% of patients with CCCA, but the exact role of this variant in the occurrence of CCCA is unclear. Furthermore, studies suggest that women with CCCA are more likely to have uterine fibroids (a noncancerous tumor of smooth muscle). Various hairstyling practices have been studied as potentially causing CCCA, but none has been definitively linked to the condition. A combination of factors such as infections (bacterial and fungal), genetics, autoimmune disease, and other unknown factors may play a role in this disease. More research is needed to understand the extent to which any or all of these factors contribute to the development of CCCA.
Symptoms and Diagnosis
At its onset, CCCA presents as hair breaking and thinning in the crown of the scalp. The area of thinning hair expands over time and progresses to more severe, permanent hair loss. Some patients experience burning, itching, tenderness, redness, scaling (flaking), and acne-like pimples or bumps in the region of hair loss.
A diagnosis of CCCA is often made through a clinical examination of the scalp. A dermatoscope, which is a handheld magnifying tool, may be used to view the affected area. The physician may also perform a scalp biopsy (a small portion of the scalp skin is removed and examined under microscopic) to support the diagnosis.
The main goal of treating CCCA is to relieve its symptoms, to slow or stop further hair loss and promote hair regrowth in areas not permanently scarred. Mild CCCA is often treated with a topical steroid medication applied by the patient at home and sometimes combined with local corticosteroid injections in the scalp performed by the physician at 4- to 8-week intervals. The treatment of more severe CCCA will combine these treatments with oral medications that treat inflammation by suppressing the immune system (immunosuppressants).
Women with CCCA should also consider changing their hair care practices. While there is no clear evidence linking hair styling to CCCA, avoiding the use of potentially damaging chemicals (relaxers should be applied by a professional only) and hair styles (tight braids, extensions, weaves) and minimizing the use of heated styling tools (hot combs, hooded hair dryers, blow dryers, flat irons) will reduce further damage. Patients are also encouraged to wash their hair at least once every 1 to 2 weeks because this helps decrease inflammation of the scalp.
Central Centrifugal Cicatricial Alopeciahttps://dermnetnz.org/topics/central-centrifugal-cicatricial-alopecia/
Central Centrifugal Cicatricial Alopeciahttps://skinofcolorsociety.org/dermatology-education/central-centrifugal-cicatricial-alopecia-ccca/
Published Online: July 29, 2020. doi:10.1001/jamadermatol.2020.1859
Conflict of Interest Disclosures: Dr Aguh reports serving as consultant for L’oreal Inc, LEO Pharma, DevaConcepts, and UCB Pharma and receiving royalties from UpToDate. Dr McMichael reports consulting for Aclaris, Allergan, Almirall, Bioniz, Cassiopea, Covance, eResearch Technology Inc, Galderma, Incyte, Johnson & Johnson, Keranetics, Merck & Co Inc, Pfizer, Proctor & Gamble, Revian, Samumed; conducting research for Aclaris, Cassiopea, Concert Pharmaceuticals, Incyte, Proctor & Gamble, and Samumed; receiving grants from Allergan, Concert Pharmaceuticals, and Proctor & Gamble; and receiving royalties from Informa Healthcare and UpToDate.
Aguh C, McMichael A. Central Centrifugal Cicatricial Alopecia. JAMA Dermatol. 2020;156(9):1036. doi:10.1001/jamadermatol.2020.1859
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