Ncoza C Dlova1, Francois H Jordaan2, Ofer Sarig3, Eli Sprecher4. 1. Dermatology Department, Nelson R. Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa. Electronic address: dlovan@ukzn.ac.za. 2. Division of Dermatology, Faculty of Health Sciences, University of Stellenbosch Tygerberg Hospital, Cape Town, South Africa. 3. Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. 4. Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
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.
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.
Authors: Patricia F Coogan; Traci N Bethea; Yvette C Cozier; Kimberly A Bertrand; Julie R Palmer; Lynn Rosenberg; Yolanda Lenzy Journal: Int J Womens Dermatol Date: 2019-06-06