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.
Keywords: African; South Africa; black; familial; follicular degeneration syndrome; genetic; hair loss; lymphocytic primary scarring alopecia; scarring alopecia.
Copyright © 2014 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.