Central centrifugal cicatricial alopecia (CCCA) nomenclature describes a typical clinical presentation of cicatricial hair loss that begins on the vertex scalp with progressive, symmetric, and centrifugal evolution. However, atypical presentations have been noted clinically by the authors and reported in the literature.

We sought to characterize the distribution of hair loss in published cases of adult patients with CCCA.

A 3-step search process was used to evaluate research articles in Cumulative Index to Nursing & Allied Health, EMBASE, Google Scholar, MEDLINE, Scopus, and Web of Science databases. Studies with scalp photography or description of hair loss distribution were included. Three researchers evaluated eligible studies for clinical subtypes. Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Review was used to report results.

Ninety-nine studies consisting of 281 cases of CCCA were included. Hair loss distributions included variants of the classic presentation along with distinct subtypes such as patchy, occipital, parietal, frontal, temporal, and trichorrhexis.

Studies had significant homogeneity, as the classic distribution of CCCA was commonly reported. Additionally, clinically diagnosed cases may have concurrent diagnoses, and numerous studies did not report trichoscopy findings.

CCCA terminology may not always be reflective of clinical presentation. Understanding atypical presentations is essential to inform appropriate and targeted treatment.