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Central centrifugal cicatricial alopecia - Hair and Scalp
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Central centrifugal cicatricial alopecia - Hair and Scalp

Contributors: Kiasha Govender MBChB, MMed, FCDerm, Vivian Wong MD, PhD, Anisa Mosam MBChB, MMed, FCDerm, PhD, Ncoza C. Dlova MBChB, FCDerm, PhD, Callyn Iwuala BA, Oyetewa Oyerinde MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Central centrifugal cicatricial alopecia (CCCA), also known as central centrifugal scarring alopecia, is a scarring alopecia predominantly affecting middle-aged women of African descent, with a prevalence of 3%-6% in this demographic.

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

Look For

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Diagnostic Pearls

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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
  • Favus
  • Trichotillomania
  • Traction alopecia (especially if long-standing)
  • Alopecia from heat or chemical burn
  • Discoid lupus erythematosus
  • Lichen planopilaris
  • Acne keloidalis nuchae

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:07/24/2019
Last Updated:09/28/2022
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Central centrifugal cicatricial alopecia - Hair and Scalp
A medical illustration showing key findings of Central centrifugal cicatricial alopecia : Burning skin sensation, Vertex scalp, Pruritus, Scarring alopecia, Short broken hairs
Clinical image of Central centrifugal cicatricial alopecia - imageId=848531. Click to open in gallery.  caption: 'A patch of scarring alopecia on the parietal scalp.'
A patch of scarring alopecia on the parietal scalp.
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