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.


CONCLUSIONS
Small number of patients is a limitation.


CONCLUSIONS
CCCA can be inherited in an autosomal dominant fashion, with partial penetrance and a strong modifying effect of hairstyling and gender.
Dermatology Department, Nelson R. Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa. Electronic address: dlovan@ukzn.ac.za.
Ncoza C
NC
Dlova
Division of Dermatology, Faculty of Health Sciences, University of Stellenbosch Tygerberg Hospital, Cape Town, South Africa.
Francois H
FH
Jordaan
Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Ofer
O
Sarig
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.
Eli
E
Sprecher
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000505:Alopecia / Q000208:ethnology / Q000235:genetics* / Q000473:pathology*; D001707:Biopsy, Needle; D002648:Child; D025063:Chromosome Disorders / Q000235:genetics; D002921:Cicatrix / Q000208:ethnology / Q000235:genetics / Q000473:pathology*; D015331:Cohort Studies; D005260:Female; D005799:Genes, Dominant / Q000235:genetics*; D020022:Genetic Predisposition to Disease / Q000453:epidemiology*; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012189:Retrospective Studies; D018570:Risk Assessment; D017678:Sex Distribution; D013019:South Africa / Q000453:epidemiology; D055815:Young Adult
African; South Africa; black; familial; follicular degeneration syndrome; genetic; hair loss; lymphocytic primary scarring alopecia; scarring alopecia