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Central centrifugal cicatricial alopecia (CCCA) is the most common form of primary scarring alopecia in women of African descent.
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At the molecular level, an autosomal dominant mode of inheritance, mutations in protein PADI3, and upregulation of critical fibroproliferative genes have all been linked to patients with CCCA.
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CCCA often occurs in the absence of clinical signs of overt inflammation and instead fibrosis is often the predominant feature.
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The presence of a white/gray peripilar follicular
Central Centrifugal Cicatricial Alopecia: Challenges and Treatments
Section snippets
Key points
Prevalence
The true prevalence of CCCA has yet to be established; however, multiple studies have been conducted to explore the prevalence in different demographics. A study completed in 2011 sought to discern the prevalence of CCCA in African American women, where patients from the central/southeast United States were recruited and their hair loss evaluated through the use of a standardized photographic scale5 and questionnaire; the study concluded a prevalence of 5.6% in a study of 529 subjects.6,7 A
Pathogenesis
The pathogenesis of CCCA is believed to involve a complex mixture of a multitude of factors including but not limited to genetic predisposition,13 variants in gene expression,14 ethnic hair care practices,15 and disruption of the balance between proinflammatory and anti-inflammatory factors. One study of 14 South African index families with 31 immediate family members found evidence to suggest that CCCA can be inherited in an autosomal dominant fashion.13
New research14 suggests that pathogenic
Associated findings
More recent literature has explored the possible connection between CCCA and systemic diseases. One study reported a statistically significant increase in the prevalence of diabetes mellitus type 2 among patients with CCCA, suggesting that CCCA may be a marker of metabolic dysregulation.8 A retrospective study reported that women with CCCA were found to have a nearly 5 times increased odds of having uterine leiomyomas compared with race-matched, age-matched, and sex-matched controls.17 This
Dermatoscopy
Dermatoscopy serves as a noninvasive diagnostic tool to elucidate specific findings seen in CCCA and to select the best sites for obtaining biopsy specimens. In a retrospective study, a highly sensitive and specific finding of a peripilar gray/white halo located around the emergence of hair follicles was observed in 94% of patients diagnosed with CCCA.18 Other reported dermatoscopic findings include honeycomb pigmented rete ridges and hypomelanotic dermal papillae, hair shaft variability,
Histopathology
Histopathologic analysis is useful in the diagnosis of CCCA and specific features can help distinguish CCCA from other scarring alopecias. The histopathology of CCCA is usually summarized by the presence of premature desquamation of the inner root sheath (PDIRS) at the level of the deep dermis with varying degrees of perifollicular lymphocytic inflammation around the infundibulum and isthmus that leads to follicular destruction and replacement of hair shafts with fibrosis.20 PDIRS is such a
Clinical presentation
CCCA typically begins as an area of decreased hair density or hair breakage notably at the vertex of the scalp and expands peripherally (Fig. 1). In early stages, patients may report scalp pruritus or tenderness. The hair loss can progress insidiously, delaying diagnosis and treatment especially in asymptomatic patients. Advanced cases reveal impressive hair loss evidenced by a smooth, shiny scalp with loss of follicular ostia (Fig. 2). Hair breakage also can be a sign of early or occult CCCA,
Patient evaluation overview
When evaluating a patient with suspected CCCA, a thorough history should be obtained from the patient. Box 1 lists pertinent questions applicable to patients with suspected CCCA. Baseline photographs of the affected area(s) are important to evaluate the patient’s condition at subsequent follow-up visits. A detailed scalp examination using adequate lighting and a dermatoscope is necessary. Dermoscopy is essential in identifying the optimal site for a biopsy and narrowing the differential
General Guidelines
The general goals in treating CCCA are aimed at addressing symptoms at the earliest presentation, halting progression of disease, and establishing hair regrowth. These steps involve patient education, topical agents, systemic medications, and in some cases, procedural therapies. Discontinuation of hair grooming practices that promote fragility, tension, and trauma to the hair follicle is essential.31 It is also important to acknowledge the psychological impact that patients with CCCA may
The role of hair care practices
The differences in hair structure in patients of African ancestry compared with other ethnic backgrounds plays an important role when discussing treatments for CCCA. In its natural state, the African hair shaft tends to be elliptical or flattened in cross-section and spiral or tightly curled in its tertiary structure.38 It is important to note that not all patients of African descent exhibit curly hair. African hair also exhibits more knots, broken hair shafts, and interlocking of hair shafts.50
Challenges and solutions
Early CCCA can often be difficult to distinguish from the most common form of alopecia, which is AGA. Unfortunately, this can pose a major challenge and delay appropriate treatment. CCCA in men can present almost identical to AGA; however, the former usually presents at an earlier age, is symptomatic, and is restricted to the vertex, whereas the latter can have asymptomatic additional hair loss in the frontal or bi-temporal hairline or crown.12 This highlights the importance in performing a
Summary
CCCA remains the leading cause of scarring alopecia in women of African descent. Although certain traumatic hairstyles play a contributory role, we now understand this is a much more complex disease process. Future research will hopefully identify the exact inheritance pattern of CCCA, other causal genes linked to CCCA, and systemic diseases that we should screen for in our patients diagnosed with CCCA. In addition, future clinical studies should explore possible therapeutic options that target
Clinics care points
CCCA is the most common form of primary scarring alopecia in women of African descent.41 At the molecular level, an autosomal dominant mode of inheritance,13 mutations in protein PADI3,14 and upregulation of critical fibroproliferative genes16 have all been linked to patients with CCCA. CCCA often occurs in the absence of clinical signs of overt inflammation and instead fibrosis is often the predominant feature.30 The presence of a white/gray peripilar follicular halo on dermoscopy is both
Disclosure
The authors have no disclosures to report.
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2022, JAAD Case ReportsCitation Excerpt :Hair loss begins at the vertex or crown of the scalp and spreads centrifugally over time. Patients often report associated symptoms of scalp burning, itching, tenderness, and scaling.6 Dermoscopy may be used to identify specific features of CCCA, including a peripilar gray/white halo around the emergence of the hair follicle, honeycomb pigmented rete ridges and hypomelanotic dermal papillae, perifollicular erythema, and hair breakage appearing as black dots on the scalp.6,7