Abstract
This cross-sectional study assesses the dermatoscopic findings 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.
Methods
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.
Results
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).
Table. Histopathologic and Dermatoscopic Findings From the Clinically Affected and Unaffected Scalp of 10 Patients With CCCA.
| Patient No. | Disease duration, y | Dermatoscopic findings | Histopathologic findings | |
|---|---|---|---|---|
| Clinically affected vertex scalp | Clinically unaffected occipital scalp | |||
| 1 | 5 | Follicular scale, interfollicular erythema, terminal and vellus hairs, peripilar white halo, honeycomb pattern | Follicular scale, peripilar white or gray halo, terminal and vellus hairs, broken hairs, interfollicular erythema | Perifollicular inflammation and moderate perifollicular fibrosis, consistent with CCCA |
| 2 | 9 | Peripilar white or gray halo, perifollicular erythema, perifollicular scale, honeycomb pattern, interfollicular hyperpigmented patches, terminal and vellus hairs, increased interfollicular distance | Peripilar white or gray halo, perifollicular scale, honeycomb pattern, terminal and vellus hairs, perifollicular erythema, increased interfollicular distance | NA |
| 3 | 11 | Peripilar white or gray halo, increased interfollicular distance, honeycomb pattern, pinpoint white dots, terminal and vellus hairs, interfollicular hyperpigmented patches | Peripilar white or gray halo, perifollicular scale, increased interfollicular distance, honeycomb pattern, pinpoint white dots, terminal and vellus hairs, interfollicular and perifollicular erythema | NA |
| 4 | 3 | Perifollicular erythema, pinpoint white dots, peripilar white gray or halo, perifollicular scale, honeycomb pattern, terminal and vellus hairs, increased interfollicular distance | Peripilar white or gray halo, perifollicular erythema, perifollicular scale, honeycomb pattern, terminal and vellus hairs | NA |
| 5 | 8 | Honeycomb pattern, peripilar white or gray halo, interfollicular erythema, hypopigmentation, broken hairs, terminal and vellus hairs, pinpoint white dots | Peripilar white or gray halo, increased interfollicular distance, honeycomb pattern, terminal and vellus hairs, pinpoint white dots | NA |
| 6 | 35 | Follicular scale, interfollicular erythema, peripilar white or gray halo, broken hairs, terminal and vellus hairs | Follicular scale, perifollicular and interfollicular erythema, terminal and vellus hairs, honeycomb pattern, peripilar white or gray halo | NA |
| 7 | 18 | Broken hairs, vellus and terminal hairs, interfollicular erythema, peripilar white halo, increased interfollicular distance, honeycomb pattern | Peripilar white or gray halo, honeycomb pattern, terminal and vellus hairs, broken hairs, interfollicular erythema | Mild perifollicular fibrosis consistent with early CCCA changes |
| 8 | 10 | Peripilar white or gray halo, peripilar dark halo, vellus and terminal hairs, follicular scale, honeycomb pattern, pinpoint white dots | Peripilar white or gray halo, perifollicular erythema, honeycomb pattern, pinpoint white dots, terminal and vellus hairs, increased interfollicular distance | Perifollicular inflammation and mild fibrosis, consistent with early CCCA |
| 9 | 8 | Perifollicular and intrafollicular erythema, terminal and vellus hairs, honeycomb pattern, perifollicular white halo | Perifollicular and interfollicular erythema, peripilar white or gray halo, perifollicular scale, terminal and vellus hairs, honeycomb pattern | Perifollicular fibrosis, focal granulomatous inflammation, consistent with early CCCA |
| 10 | 6 | Peripilar white or gray halo, vellus and terminal hairs, follicular scale, honeycomb pattern, broken hairs, interfollicular erythema | Peripilar white or gray halo, vellus and terminal hairs, follicular scale, broken hairs, interfollicular erythema | NA |
Abbreviations: CCCA, central centrifugal cicatricial alopecia; NA, not applicable.
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.
Figure. Findings of Central Centrifugal Cicatricial Alopecia.
A, Biopsy site (circle) on the clinically unaffected occipital scalp. B, Histopathologic findings from posterior scalp include perifollicular fibrosis and lymphocytic infiltration (hematoxylin-eosin, original magnification ×20). C, Dermatoscopic findings include peripilar white gray halo and interfollicular erythema.
Discussion
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.
References
- 1.Ogunleye TA, McMichael A, Olsen EA. Central centrifugal cicatricial alopecia: what has been achieved, current clues for future research. Dermatol Clin. 2014;32(2):173-181. Published online January 22, 2014. doi: 10.1016/j.det.2013.12.005 [DOI] [PubMed] [Google Scholar]
- 2.Miteva M, Tosti A. Central centrifugal cicatricial alopecia presenting with irregular patchy alopecia on the lateral and posterior scalp. Skin Appendage Disord. 2015;1(1):1-5. Published online January 7, 2015. doi: 10.1159/000370315 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Olsen EA, Callender V, Sperling L, et al. Central scalp alopecia photographic scale in African American women. Dermatol Ther. 2008;21(4):264-267. doi: 10.1111/j.1529-8019.2008.00208.x [DOI] [PubMed] [Google Scholar]
- 4.Uhlenhake EE, Mehregan DM. Prospective histologic examinations in patients who practice traumatic hairstyling. Int J Dermatol. 2013;52(12):1506-1512. Published online March 3, 2013. doi: 10.1111/j.1365-4632.2012.05621.x [DOI] [PubMed] [Google Scholar]
- 5.Miteva M, Tosti A. Hair and scalp dermatoscopy. J Am Acad Dermatol. 2012;67(5):1040-1048. Published online March 8, 2012. doi: 10.1016/j.jaad.2012.02.013 [DOI] [PubMed] [Google Scholar]
- 6.Miteva M, Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia. J Am Acad Dermatol. 2014;71(3):443-449. Published online June 11, 2014. doi: 10.1016/j.jaad.2014.04.069 [DOI] [PubMed] [Google Scholar]