Autosomal dominant inheritance of central centrifugal cicatricial alopecia in black South Africans

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. © 2014 by the American Academy of Dermatology, Inc.