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AUTOSOMAL DOMINANT INHERITANCE OF CENTRAL CENTRIFUGAL CICATRICIAL ALOPECIA IN BLACK SOUTH AFRICANS

Abstract

Background: Central centrifugal cicatricial alopecia (CCCA) is the commonest type of primary scarring alopecia in women of African descent. Little is currently known about the disease genetics.

Objective: We sought to investigate patterns of inheritance in CCCA and ascertain the contribution of nongenetic factors such as hair-grooming habits to the pathogenesis of the disease.

Methods: Affected individuals with at least 1 available family member were recruited from 2005 through
2012 inclusive for pedigree analysis. CCCA was diagnosed on clinical and histopathological grounds.

Results: Fourteen index African families with 31 immediate family members participated in the initial screening. The female to male ratio was 29:2 with an average age of 50.4 years. All patients displayed histologic features typical for CCCA. Pedigree analysis suggested an autosomal dominant mode of inheritance. Hair-grooming habits were found to markedly influence disease expression.

Limitations: Small number of patients is a limitation.

Conclusion: CCCA can be inherited in an autosomal dominant fashion, with partial penetrance and a strong modifying effect of hairstyling and gender. ( J Am Acad Dermatol 2014;70:679-82.)

Keywords: African; black; familial; follicular degeneration syndrome; genetic; hair loss; lymphocytic primary scarring alopecia; scarring alopecia; South Africa.

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