Dermatoscopic Evaluation of Central Centrifugal Cicatricial Alopecia Beyond the Vertex Scalp

Central centrifugal cicatricial alopecia (CCCA) is a scarring alopecia commonly seen in African American individuals.¹ It classically affects the vertex scalp, although 1 study² suggests that it may manifest as patchy hair loss beyond the vertex. In that study, the histopathologic, dermatoscopic, and clinical findings were crucial in diagnosis. However, another study³ reported histopathologic changes in the vertex scalp consistent with CCCA even without clinically visible hair loss. Our study explored dermatoscopic and histopathologic findings in areas of the scalp without clinically active disease, specifically beyond the vertex scalp. We also assessed the reliability of dermatoscopy, which may serve as a less invasive diagnostic tool than biopsy, for the diagnosis of CCCA in the context of subclinical disease.

For this cross-sectional study, African American women (aged ≥18 years) with CCCA diagnosed on the basis of clinical and histopathologic findings (central scalp alopecia photographic scale severity 2-5 [range, 0-5, with higher scores indicating worse alopecia]) were recruited at dermatology clinics from June 18, 2018, to August 13, 2018.⁴ Patients with additional inflammatory scalp disease and those who received intralesional steroids or systemic therapy for CCCA within the past 6 months were excluded. Clinical photographs of the posterior, vertex, and bilateral parietotemporal scalp were obtained. Dermatoscopic photographs were obtained of the clinically active edge of hair loss and 4 cm peripherally, toward the central posterior and bilateral parietooccipital scalp. Dermatoscopic images were evaluated by a single evaluator (A.J.M), who was blinded to scalp location and clinical appearance, for common findings in CCCA as described by Miteva and Tosti.^5,6 This study was approved by the institutional review board at Wake Forest Baptist Medical Center, Winston Salem, North Carolina. Written consent was obtained from all study participants.

Four patients underwent two 4-mm punch biopsies of the clinically unaffected posterior scalp. Histopathologic findings were described by a single evaluator (N.P.), who was blinded to dermatoscopic and clinical findings. Each patient completed a survey evaluating scalp symptoms. Descriptive statistical analysis was completed between August 13 and August 18, 2018, using Microsoft Excel 2016 software.

Ten women (mean [SD] age, 48.7 [11.5] years) were enrolled. The mean (SD) self-reported severity score was 3.9 (1.3).⁴ Patients reported hair loss from the root within the vertex (10 of 10 patients [100%]) and beyond the vertex (4 of 10 [40%]). Most patients reported itching within the vertex (8 of 10 [80%]) and regions outside the vertex (6 of 10 [60%]). Breakage of the hair was common in the vertex (7 of 10 [70%]) and outside the vertex (7 of 10 [70%]). Tenderness was reported mostly in the vertex (8 of 10 [80%]) as opposed to beyond the vertex (1 of 10 [10%]). Most patients had undergone previous treatment (9 of 10 [90%]) and reported improvement of symptoms with topical steroids (5 of 8 [62%]), intralesional steroids (6 of 10 [60%]), and topical minoxidil (4 of 9 [44%]).

Classic dermatoscopic findings of CCCA were visualized on all patients throughout the 4 observed scalp regions, including (1) peripilar white or gray halo (38 of 40 patients [95%]), (2) honeycomb patterns (32 of 40 [80%]), (3) perifollicular and/or interfollicular erythema (33 of 40 [82%]), (4) follicular scale (27 of 40 [68%]), (5) terminal and vellus hairs (34 of 40 [85%]), (6) increased interfollicular distance (14 of 40 [35%]), (7) broken hairs (15 of 40 [38%]), (8) pinpoint white dots (10 of 40 [25%]), and (9) interfollicular hyperpigmented patches (3 of 40 [7.5%]). Dermatoscopic findings in clinically unaffected and clinically affected sites were mostly identical (Table).

Histopathologic findings were also consistent with early CCCA (Table and Figure). In the 4 scalp biopsies performed outside clinically evident disease, perifollicular fibrosis and lymphocytic infiltration were seen, possibly associated with the dermatoscopic findings of peripilar white or gray halo and erythema, respectively.

Although the current paradigm for CCCA treatment is guided by the clinically visible edge, clinicians may omit surrounding areas of early, subclinical disease by treating focally. Our study findings suggest that dermatoscopic and histopathologic evidence of CCCA can be seen beyond the vertex scalp, even without clinically evident disease in those areas. This finding may warrant the use of dermatoscopic examination to identify the extent of disease as well as the areas in need of scalp-directed treatment. Small sample size was a limitation to this study.

Further associations between key histopathologic and dermatoscopic features are needed to determine whether dermatoscopic examination may replace the need for biopsy as part of the diagnostic evaluation and clinical care of this patient population. This may ultimately allow for less-invasive diagnosis, optimized treatment margins, improved patient outcomes, and delayed disease progression.

Corresponding Author: Kayla Felix, MS, Wake Forest School of Medicine, 4618 Country Club Rd, Winston-Salem, NC 27104 (kfelix@wakehealth.edu).

Published Online: July 1, 2020. doi:10.1001/jamadermatol.2020.1287

Author Contributions: Dr McMichael had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Concept and design: Felix, De Souza, Sangueza, McMichael.

Acquisition, analysis, or interpretation of data: Felix, De Souza, Portilla, Hogue, Ahn, McMichael.

Drafting of the manuscript: Felix, Ahn, Sangueza, McMichael.

Critical revision of the manuscript for important intellectual content: De Souza, Portilla, Hogue, Sangueza, McMichael.

Statistical analysis: Portilla.

Administrative, technical, or material support: Felix, De Souza, Portilla, Hogue.

Supervision: De Souza, Ahn, Sangueza, McMichael.

Conflict of Interest Disclosures: Dr McMichael reported being a consultant for Almirall, Galderma, Incyte, and Procter & Gamble; reported performing research for Incyte and Procter & Gamble; reported receiving grants from Procter & Gamble; and reported receiving personal fees from UpToDate outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.