What Is Central Centrifugal Cicatricial Alopecia?

Overview

Hair loss (alopecia) and balding are issues that concern a significant portion of the human population and can have a detrimental impact on people’s mental health and overall well-being. While the scientific community recognises multiple types of hair loss, successful treatments can be more complex and occur for an extended period. 

Central centrifugal cicatricial alopecia (CCCA) is a specific type of alopecia characterised by an initial hair loss in the scalp’s centre (vertex or crown). This hair loss gradually expands and may eventually result in permanent hair loss with scar tissue formation (fibrosis). This condition is most frequently observed in Black people assigned female at birth (AFAB), with the affected patients presenting varying clinical symptoms. Unfortunately, an adequate treatment for this condition is rather challenging. However, prevention (by limiting hair-damaging practices and substances) and early diagnosis (to stop the balding progression) are key factors in addressing this disease in the most efficient manner.¹

Introduction

Alopecia derives from the Greek word ‘alopex’, which means fox, and was historically associated with the fur loss detected in foxes suffering from mange. It is a condition that has perplexed physicians for centuries, as the term itself refers back to the time of Hippocrates.² Current literature has allowed us to conceive as thoroughly as possible the aetiology, symptoms, and treatment options for this condition. 

It’s more convenient to briefly mention some common forms of alopecia to accurately differentiate between them. The most prevalent forms include male androgenetic alopecia (MAA), female androgenetic alopecia (FAGA), alopecia areata (AA), CCCA, anagen effluvium and telogen effluvium.

CCCA belongs to the group of primary lymphocytic inflammatory cicatricial alopecias, and as the name indicates, it begins with an initial hair loss on the crown of the scalp that may progress further, potentially destroying hair follicles and the formation of scar tissue. However, other clinical presentations have also been noted, including patchy hair loss (both centrally and laterally), on the scalp of patients.³ CCCA is most prevalent in Black people AFAB, without entirely excluding its incidence in both sexes and other ethnicities. 

Up-to-date epidemiologic studies provide narrow numerical data for CCCA because of the limited populational association with the disease. However, currently, available data suggests a prevalence of CCCA ranging from 2.7% to 5.7% in people AFAB of African ancestry, with the average age of onset of the disease around 36 years.^4,5

This article aims to enhance the understanding of the clinical image, implications and potential therapeutic approaches for this condition. 

Causes and risk factors

Hair care practices and grooming habits

In the past, it was markedly suggested that specific hair practices, styling methods, and the use of harsh chemicals on hair were primarily associated with CCCA. Recent studies, however, have rejected an immediate correlation between the two, as other more critical factors have come into play. For instance, CCCA was also known as ‘hot comb alopecia’, and was hypothesised to be caused principally by the use of a hot comb and petroleum jelly - a technique that is commonly used by Black people AFAB to straighten their hair.

Subsequent research contradicted this belief, however, as it included people AFAB with similar clinical presentations who did not follow the same hair care practices. Eventually, the term CCCA prevailed and is used to refer to conditions that are described by expanding follicular degeneration and fibrosis.⁶

In summary, although there is no verified link between specific hair care methods and products and CCCA, specific studies have been posted investigating further association. A review of dermatological reports over 5 years did reveal a correlation between hairstyle practices and CCCA, as well as with treatment outcomes. Precisely, it was stated that the use of chemical relaxers on the hair was affiliated with CCCA. Additionally, the use of hooded dryers seemed to increase the likelihood of improvement, while unrelaxed hairstyles (e.g., tight braids and extensions) appeared to decrease positive treatment outcomes. Prospective studies exploring more in-depth hair practices and CCCA are required for a more profound interpretation.⁷

Genetic predisposition

CA, as mentioned earlier, is most frequently observed in people AFAB of African descent and may be a hereditary condition affecting multiple family members. This indicates the presence of a genetic trait that could be responsible, among other factors, for the disease’s occurrence. In fact, a mutation in the PADI3 gene, which encodes the enzyme peptidyl arginine deiminase, type III, has been identified. The enzyme PADI3 plays a crucial role in the production of proteins fundamental for the formation of the hair shaft, the visible part of the hair.

Specifically, a study conducted among a group of people AFAB with alopecia suggests that the disease may follow an autosomal dominant inheritance pattern, meaning that a single copy of the mutated gene PADI3 may result in CCCA. However, CCCA is considered a multifactorial disorder where the variations in hair structure among people from diverse ancestries and the presence of other conditions such as bacterial or fungal infections or autoimmune diseases may also contribute to the disease onset and a potentially biased clinical image.⁸

Clinical presentation

Symptoms and early signs

While CCCA’s aetiology remains challenging, its symptoms may also present ambiguity. In general, patients initially experience hair loss in the centre of the scalp, which then progresses towards the sides. Further symptoms include:

• Some patients may exhibit evident hair loss on the vertex of the scalp but remain completely asymptomatic.
• Hair breakage, particularly in the central part of the scalp, may be associated with early signs of CCCA.
• Other patients may experience pain, tenderness, burning and itchy sensations on the scalp.
• People may also notice unusual sensations due to the inflamed state of their scalp, with the skin becoming crusty and developing small bumps even before apparent hair loss. Specifically, the presence of seborrheic scales ­(scaly patches), follicular papules (inflamed bumps), and pustules (bulging patches that usually contain pus­­) have been observed in patients, eventually resulting in damage to the follicular ostia ­(openings in the skin’s surface where hair follicles are located).⁵
• In most cases, the bald patch of skin may feel smooth and appear shiny. According to dermatologists, this is likely due to the destruction of hair follicles and the formation of scar tissue.

Progression and diagnosis

The severity of CCCA in patients who have sought medical assistance is usually evaluated using a photographic scale.⁵ Early symptoms such as itching and tenderness might develop before hair loss, leading to delays in diagnosis and treatment. This is also the case for asymptomatic patients.⁹ The symptoms of the later stages of this disease may develop slowly, over decades, or more rapidly over the years.

The diagnosis of CCCA should commence with healthcare professionals obtaining a detailed clinical history from patients. Continuous hair care practices and the use of chemicals should be mentioned. Patients should also provide details of their medication history, including any newly prescribed drugs, oral contraceptives and hormonal modifiers.¹⁰

The diagnostic procedure involves a dermatological examination (dermatoscopy) to identify the most appropriate site (usually a grey halo located near the hair follicles indicating a state of inflammation) from which biopsy specimens are collected and subjected to examination.^5,9

Management and treatment

The most efficient way to manage this condition is to obtain an early diagnosis to maintain symptom stability and prevent further hair loss. Unfortunately, in the advanced stages of CCCA, the centrifugal bald patches are typically irreversible due to the destruction of hair follicles and the presence of fibrosis. 

Treatment may alleviate symptoms, suppress the general inflammation observed in the affected area, and halt the disease progression. Therapeutic solutions include

• A shift towards non-invasive hair care routines. The motive is to relieve scalp tension that might cause hair breakage but also minimise the use of heavy chemicals for hair care purposes.¹⁰
• Oral medications are advised in patients with active progression of the disease to diminish skin inflammation. Specific options include anti-inflammatory drugs and antibiotics such as tetracycline and doxycycline or antimalarial drugs such as hydroxychloroquine due to their anti-lymphocytic activity.¹⁰
• Topical or injected corticosteroids and intralesional injections are also considered to mitigate local symptoms on the scalp.¹⁰ Minoxidil may also be used as an additional therapy.
• Surgical treatment may be advised in certain circumstances, though it bears several challenges due to the difficulty of successfully transplanting healthy hair grafts into scar tissue where blood flow is limited.⁵
• Recent reports stated that several patients were treated with a botanical formulation and exhibited an improvement attributed to the anti-inflammatory and fibrotic-diminishing properties of this novel therapy.¹¹

Summary

In conclusion, CCCA is a complex challenge not only for those affected but experts, too. Early diagnosis is vital to prevent irreversible hair loss, underscoring the importance of awareness and routine scalp examinations. Yet, our understanding of CCCA remains incomplete, necessitating further research to develop personalised therapies. Seeking professional help is crucial for diagnosis and guidance, while support groups provide invaluable emotional and practical assistance. By promoting awareness, early detection, research, and professional support, significant strides can be made in addressing CCCA's impact on individuals’ lives and in working towards improved treatments and outcomes.