Central centrifugal cicatricial alopecia - Hair and Scalp
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Synopsis
The exact pathogenesis of CCCA is unknown, but its cause is likely multifactorial. A genetic defect in the internal root sheath in patients of African descent, inherited in an autosomal dominant manner, has been suggested. Mutations in PADI3, which encodes a protein that is essential for normal hair-shaft formation, have been shown to be associated with CCCA. Triggering or aggravation of the disease may then occur following traumatic hair care practices, such as cornrows and braiding, extensions, weaves with sewn-in or glued-on hair, use of hot combs, and frequent use of hair relaxers. Other factors implicated in the pathogenesis, but never conclusively proven, include scalp infections and type 2 diabetes mellitus.
Classically, CCCA presents with progressive hair loss, starting as a single patch at the vertex of the scalp and then expanding in a centrifugal and symmetrical pattern. Unusual variants with multiple irregular patches of hair loss have been described. Although not typical, follicular papules, scaling, erythema, and pustules may occasionally be seen. Initially, scarring may be subtle, but as the disease progresses, it culminates in shiny, scarred plaques. Some residual hairs may be found throughout the involved area.
CCCA is usually symptomatic and may be accompanied by burning, dysesthesia, and/or pruritus.
CCCA was formerly referred to as hot comb alopecia and may also be known as follicular degeneration syndrome, pseudopelade in African Americans, and scarring alopecia in African Americans.
Codes
ICD10CM:L66.8 – Other cicatricial alopecia
SNOMEDCT:
109441000119102 – Central centrifugal cicatricial alopecia
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Differential Diagnosis & Pitfalls
- Female pattern alopecia – Diffuse thinning of the crown and widening of the midline part with retention of the frontal hairline. In female pattern hair loss, the part is wider at the vertex than it is at the occiput.
- Male pattern alopecia – Follows a frontal / temporal or vertex pattern of hair loss.
- Alopecia areata
- Telogen effluvium
- Tinea capitis (especially Trichophyton tonsurans)
- Pseudopelade of Brocq
- Favus
- Trichotillomania
- Traction alopecia (especially if long-standing)
- Alopecia from heat or chemical burn
- Discoid lupus erythematosus
- Lichen planopilaris
- Folliculitis keloidalis
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Last Reviewed:07/24/2019
Last Updated:09/28/2022
Last Updated:09/28/2022
Patient Information for Central centrifugal cicatricial alopecia - Hair and Scalp
Overview
Central centrifugal cicatricial alopecia (CCCA) is a progressive, inflammatory, scarring alopecia that presents with hair loss originating on the center (crown) of the scalp that gradually widens. While it was formerly known as chemically induced cosmetic alopecia, follicular degeneration syndrome, and hot comb alopecia, those terms have since been replaced by CCCA because the disease has not been proven to be caused only by specific hair care practices.While the exact causes of CCCA are unclear, it is thought to be due to a combination of factors, including an underlying genetic predisposition that is triggered by traumatic hair care practices. Other factors that have been implicated but not conclusively proven include scalp infections and type 2 diabetes mellitus.
Treatment is primarily aimed at stopping the progression of the disease and saving the hair follicles with topical corticosteroids, corticosteroid injections into the scalp, or oral antibiotics, as well as avoiding chemical processing and heat or traction-inducing hairstyles (those that cause pulling of the hair). Hair transplant is an option in the case of limited scarring.
Because of the initially slow progression and location of the hair loss, many affected by CCCA might be unaware of it unless it is pointed out to them or it becomes severe. Therefore, individuals often present to a dermatologist with a late stage of CCCA, suffering from significant and permanent hair loss. Many of the complications of CCCA stem from this late diagnosis.
Treatment has been shown to be most effective in those with signs of active disease, such as inflammation and ongoing hair loss. In these earliest stages of disease, hair follicles may recover after treatment and allow for hair regrowth. However, once CCCA progresses to the scarring stage, the hair follicles are permanently damaged. At this stage, treatment is focused on limiting the progression of the disease. Thus, early diagnosis and treatment are key to maximizing the likelihood of successful treatment.
While men and children are rarely affected, it is important to screen family members of affected individuals given that there may be an underlying genetic predisposition present.
Who’s At Risk
CCCA predominantly affects Black women, with an estimated prevalence of 3%-6% and an average age of onset of 36 years in that demographic. CCCA is the most common form of scarring alopecia (irreversible hair loss) in people of African descent.Signs & Symptoms
CCCA typically begins with increased hair breakage or thinning at the crown of the scalp. While hair loss can be the only sign, it can also be accompanied by small red or white acne lesions around the hair follicles, scaly skin, itchiness, pain, tenderness, or a "pins and needles" sensation on the scalp. As it progresses, the hair loss expands outwardly and reveals a shiny, smooth scalp.Self-Care Guidelines
- Avoid hair styling that uses heat and chemical straightening, as doing so will help to prevent further damage to the hair and scalp.
- Cosmetic measures such as wigs or hair powder are effective in masking the hair loss.
- Anti-dandruff shampoo can be used to address the itchiness and flakiness sometimes associated with CCCA.
When to Seek Medical Care
See a doctor in the case of:- Any unexplained hair breakage or shedding. It may be an early sign of CCCA, even in the absence of other symptoms.
- Difficulty retaining hair length at the crown.
- Symptoms such as burning, itchiness, tenderness, or a "pins and needles" sensation on the top of the scalp.
Treatments
- Your physician may do a biopsy (remove a small piece of tissue for examination under a microscope) to confirm that there is scarring.
- Mild-to-moderate CCCA responds well to topical daily corticosteroids such as clobetasol.
- In cases of active inflammation or a lack of response to topical treatment alone, oral anti-inflammatory antibiotics such as doxycycline may be prescribed, as may intralesional steroid injections. These medications should be continued until both hair loss and inflammation are stabilized, which can take years.
- Severe CCCA that does not respond to standard therapy may be treated with hydroxychloroquine or immunosuppressants to regulate the immune response.
- In advanced cases and once disease activity has resolved through treatment, hair transplantation is an option for individuals who have experienced scarring.
Central centrifugal cicatricial alopecia - Hair and Scalp