Central Centrifugal Cicatricial Alopecia

Description

Central centrifugal cicatricial alopecia (CCCA) is the most common type of primary scarring alopecia affecting women of African ancestry, with an estimated prevalence of 2.7 to 5.6%. It may be triggered by hair-grooming habits; however, familial occurrence has been reported. Mean age at presentation is 36 years. The first sign is often unexplained hair breakage, followed by hair thinning, primarily involving the vertex scalp and progressing centrifugally. Histopathologic examination shows varying degrees of lymphocytic inflammation, follicular degeneration, and fibrosis (Malki et al., 2019).

Clinical Features

Gathers and Lim (2009) reviewed published studies of CCCA, noting that it was first reported as 'hot comb alopecia' in 1968. Other terminology included 'follicular degeneration syndrome (FDS),' 'chemically induced cosmetic alopecia,' and 'scarring alopecia in African Americans.' Eventually the descriptive designation 'central centrifugal cicatricial alopecia,' believed to encompass the previously described entities, was proposed by the North American Hair Research Society in 2001. The disorder appeared to occur almost exclusively in persons of African ancestry, predominantly in females. Commonly reported histopathologic features included lymphocytic folliculitis, perifollicular granulomatous inflammation with hair shaft foreign body giant cells, prominent destruction of the folliculosebaceous units with scar tissue fibrosis at sites of former hair follicles, and retention of arrector pili muscles. The etiology of CCCA was hypothesized to be related to the lower tensile strength and relative brittleness of African hair, in association with irritation or inflammation from hair-care processes such as thermal or chemical hair straightening, as well as excessive traction from braids and weaves.

Ogunleye et al. (2014) reviewed published research on CCCA, noting that the pathogenesis was unclear and that current evidence did not support a strong causal association with traction and/or the use of relaxers. Because 2 families had been reported in which multiple members were affected, including at least 1 affected member in each family without a history of chemical or mechanical trauma to the hair, the authors suggested that there might be a genetic defect as a primary pathologic event, with triggering or exacerbation of the disease by traumatic hair practices.

Malki et al. (2019) studied 16 women of African ancestry who were clinically diagnosed with moderate to severe CCCA. Examination revealed hair loss over the crown, with centrifugal spread and a perifollicular grayish halo on dermoscopy. Biopsy showed decreased hair follicle density and perifollicular lymphocytic infiltration with areas of fibrosis.

Inheritance

Malki et al. (2019) reported 2 families with affected mothers and daughters, consistent with a dominant mode of inheritance.

Molecular Genetics

For discussion of a possible association between CCCA and variation in the PADI3 gene, see 606755.