Central Centrifugal Cicatrizing Alopecia (CCCA )

In recent years  there has been increasing and ongoing debate in the media about the visibility of black women’s hair in its natural form. This follows Lupita Nyongo the famous black African actress getting Grazia magazine to apologize for removing her natural African textured pony tail from the front cover of its November 2017 edition. This followed her commentary on Instagram that her image had been altered to a more euro centric hair style without her consent. This episode was a reminder of the societal pressures that black women are under to alter the texture of their hair.

The application of heat, hairstyles that pull too tightly on the scalp and harsh chemical treatments that damage the hair shaft are associated with the pathogenesis of central centrifugal cicatrizing alopecia (CCCA). This is the most common form of scarring (potentially permanent) form of scarring alopecia in women of African descent.  The exact prevalence is unknown, but it is thought to vary from 2.7% to 5.7% and increases with age, most often presenting in black women after the age of 30.   It has been reported that CCCA patients are among the top 5 reasons why African-Americans seek the services of a dermatologist. Unfortunately delayed presentation and treatment is an issue, significantly impacting on quality of life and self-esteem.

It presents as hair thinning, mostly affecting the vertex (top) of the scalp. Hair loss progresses outwards (centrifugally).  Hair breakage can be an early sign. Tenderness, itch and burning may be a feature. Cosmetically it can be extremely disfiguring, impacting significantly on quality of life. It has been reported that CCCA patients are among the top five reasons why African- Americans seek dermatological evaluation.  In the UK the condition is generally under-recognised in General Practice which is due in part to the absence of teaching about the ethnic hair disorders in the curriculum.  At the time of writing there was also an absence of  content on the condition on the website of the British Association of Dermatologists website (an important source of information for doctors about hair, skin and nail conditions) and on other websites such as www.nhs.uk and www.patient.info .  There are also currently no UK guidelines on the condition.

It has been shown in a small series that CCCA can be inherited in an autosomal dominant fashion, with a partial penetrance and a strong modifying effect of hairstyling and sex.  In 2019 the discovery that mutations in the  PADI3  gene which encodes a protein that is essential for hair shaft formation was made in South Africa and published by Professor Ncoza Dlova and colleagues.

Early identification is crucial because of the progressive nature of the condition. The aim of treatment is to alleviate symptoms, halt disease progression and possibly stimulate regrowth of viable hair follicles. However current treatment recommendations are largely based on mainly anecdotal evidence. Well-designed randomized controlled trials are needed to discover optimal management.

The role of the trichologist is to recognise and advise a prompt referral to a dermatologist, which in the UK would need to go through a GP in the NHS.   Unfortunately waiting times are usually currently long.
A reasonable regime started in primary care while awaiting a dermatology appointment would include mild to high potency topical steroids, antifungal shampoo and tetracycline antibiotics. It would also be prudent to take scalp scrapings for mycology as the results may take about a month to come back. Minoxidil 2% or 5% can be added to extend the anagen phase of residual hair follicles.

Dermatologist input is crucial for scalp biopsy to confirm the diagnosis and to offer early treatment with intra and peri-lesional steroids. The approach will vary from dermatologist to dermatologist so it is important to seek out dermatologists with an interest in this condition if possible. Some dermatologists may use potent medications not available for prescription in General Practice such as Hydroxychloroquine, Mycophenolate or even Methotrexate.

As well as the medical input black women should be supported in accessing good camouflage of the hair loss including cosmetic products, wigs or bespoke hair replacement systems.

There is still a great deal of work to do in terms of raising the awareness of this condition and increasing the evidence base on treatments.  
There may be some hope on the horizon though.  
– A trial is currently underway looking at the possibility that  wounding  the area of scarring alopecia in CCCA, using a fractionated CO2 laser in combination with retinoid acid, will induce hair follicle regeneration(https://clinicaltrials.gov/ct2/show/study/NCT03491267).  The most recent update was posted in January 2020 and the results are awaited with interest.
– Case  studies of 2  patients with CCCA experienced visible hair regrowth when metformin 10% compounded in Lipoderm (PCCA, Houston, TX), a cream that optimizes transcutaneous absorption, was applied topically.

“The disease can take several years of progression to burn out.  Hair transplantation is an option once the hair loss from the disease has stabilised.”

Dated June 2020

Dr Ingrid Wilson MBChB BSc(Hons) DRCOG MPH FFPH MTTS IAT https://linktr.ee/CreweAnd

Medical Member of The Trichological Society