INTRODUCTION
Central centrifugal cicatricial alopecia (CCCA) is a common cause of progressive, permanent, scarring alopecia. It is an inflammation-induced scarring type of hair loss that begins at the vertex of the scalp and progresses centrifugally. The etiology appears to be multifactorial. It occurs in all races but primarily among persons of African descent1 and with a much greater frequency among women.2,3 The prevalence is unknown, but may vary from 2.7% in South Africa to 5.6% in the United States within this population and increases with age.2,4
Pedigree analysis suggests an autosomal dominant mode of inheritance; however, hair grooming habits may markedly influence disease expression.5 Recently, a PADI3 gene mutation has been identified in patients with CCCA.6 The PADI3 gene is expressed in the inner root sheath, and encodes a protein that is necessary in the proper development of the hair shaft.6,7 Abnormal inner root sheath desquamation has been linked to the pathogenesis of CCCA.7 This finding may provide a causal relationship or increased susceptibility to hair loss in these individuals.6 Although an association between CCCA and the use of hair grooming styles that cause traction such as sewn-in hair weaving and cornrow or braided hairstyles has been reported,8,9 discontinuing these hair styles does not stop progressive hair loss. The results of a survey designed to determine risk factors for CCCA among African American women (N=326) revealed 59% had advanced central hair loss with clinical signs of scarring.10 Among those with CCCA, the incidence of bacterial scalp infections and diabetes mellitus type 2 were significantly higher as were hair styles associated with traction.10
Early diagnosis and treatment are essential in stopping or slowing the progression of scarring and permanent hair loss. Dermoscopy and histologic evaluation may reveal early or late findings that can help establish the diagnosis.11,12 Prompt and appropriate treatment is essential to help halt or slow disease progression.13 As the likelihood of scarring is related to the extent of inflammation, anti-inflammatory medications are the mainstay of treatment, with topical and intralesional corticosteroids being first-line treatments for CCCA.2,14–17 Other treatments include topical calcineurin inhibitors, oral antibiotics, hydroxychloroquine, and hair transplantation.1,2,14,15,18–20 In addition to a discussion on camouflage techniques, patients should be counseled to avoid physical and chemical trauma to the scalp.21
Pedigree analysis suggests an autosomal dominant mode of inheritance; however, hair grooming habits may markedly influence disease expression.5 Recently, a PADI3 gene mutation has been identified in patients with CCCA.6 The PADI3 gene is expressed in the inner root sheath, and encodes a protein that is necessary in the proper development of the hair shaft.6,7 Abnormal inner root sheath desquamation has been linked to the pathogenesis of CCCA.7 This finding may provide a causal relationship or increased susceptibility to hair loss in these individuals.6 Although an association between CCCA and the use of hair grooming styles that cause traction such as sewn-in hair weaving and cornrow or braided hairstyles has been reported,8,9 discontinuing these hair styles does not stop progressive hair loss. The results of a survey designed to determine risk factors for CCCA among African American women (N=326) revealed 59% had advanced central hair loss with clinical signs of scarring.10 Among those with CCCA, the incidence of bacterial scalp infections and diabetes mellitus type 2 were significantly higher as were hair styles associated with traction.10
Early diagnosis and treatment are essential in stopping or slowing the progression of scarring and permanent hair loss. Dermoscopy and histologic evaluation may reveal early or late findings that can help establish the diagnosis.11,12 Prompt and appropriate treatment is essential to help halt or slow disease progression.13 As the likelihood of scarring is related to the extent of inflammation, anti-inflammatory medications are the mainstay of treatment, with topical and intralesional corticosteroids being first-line treatments for CCCA.2,14–17 Other treatments include topical calcineurin inhibitors, oral antibiotics, hydroxychloroquine, and hair transplantation.1,2,14,15,18–20 In addition to a discussion on camouflage techniques, patients should be counseled to avoid physical and chemical trauma to the scalp.21
