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

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Contributors: Kiasha Govender MBChB, MMed, FCDerm, Vivian Wong MD, PhD, Anisa Mosam MBChB, MMed, FCDerm, PhD, Ncoza C. Dlova MBChB, FCDerm, PhD, Susan Burgin MD
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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. CCCA is rare in men and children. There have been rare familial cases, and therefore children of affected individuals should have careful examination of their scalps.

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 from recent studies. 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 asymptomatic, but tenderness, burning, dysesthesia, and pruritis have been described occasionally.

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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Last Reviewed: 07/25/2019
Last Updated: 07/26/2019
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Central centrifugal cicatricial alopecia - Hair and Scalp
See also in: Overview
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Central centrifugal cicatricial alopecia : Burning skin sensation, Vertex scalp, Pruritus, Scarring alopecia, Short broken hairs
Clinical image of Central centrifugal cicatricial alopecia
A patch of scarring alopecia on the parietal scalp.
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