History and findings of a clinical examination-that help to diagnose central centrifugal cicatricial alopecia include:

• CCCA usually starts at the vertex of the scalp.[10]
• It progresses centrifugally, usually in a symmetrical fashion.
• The scalp is soft on palpation. 
• There can be mild hyperpigmentation seen around the hair follicles.[11]
• The affected patch of alopecia slowly blends with the surrounding normal scalp. 
• There may be mild burning, tenderness, or itching confined to the area of hair loss.
• Islands of unaffected hairs with polytrichia can be seen within affected areas.

A suspicion of central centrifugal alopecia should be considered in every case of alopecia involving the vertex in female patients of Afro-Caribbean descent.[12]. The trichoscopy shows perihilar white halos, loss of follicular openings, disrupted pigmented network, irregularly distributed pinpoint white dots, and individual broken hair follicles. The hair shaft quality can be assessed by checking the hair length, diameter, and breakage. The blunt tip of hair with broomstick ends suggest breakage or trichorrhexis nodosa. Longitudinal splitting of the distal end of hair and trichoptilosis are suggestive of hair damage secondary to heat or chemical processes.

A hair pull test needs to be performed to rule out telogen effluvium. This is a test usually yields 2 to 5 telogen hairs on pulling 50 to 100 hairs in a normal adult, but it may be increased up to 3 to 5 times in telogen effluvium. Signs of hyperandrogenism like acne, hirsutism, prolongation of menstrual cycles, and obesity need to be noted as they are associated with female pattern hair loss.[13] A serum total and free testosterone dehydroepiandrostenedione sulfate (DHEAS), prolactin levels, and luteinizing hormone, follicular stimulating hormone ratio helps in ruling out polycystic ovarian syndrome associated with female pattern hair loss.

An extreme elevation in androgens is suggestive of a virilizing tumor. Further, nutritional deficiencies need to be ruled out. This can be done by ascertaining the levels of vitamin D, serum iron, ferritin, and total iron-binding capacity. A baseline complete blood counts, renal function test, liver and thyroid function tests, syphilis serology, and antinuclear antibody screening helps in ruling out other minor causes of hair loss. An absence of hyphae in KOH mount from the scalp scraping may help in ruling out tinea capitis. Finally, two 4-mm punch biopsies (for vertical and horizontal sectioning) from the active margin of the alopecia may help in the confirmation of the diagnosis.[4]

Treatment aims include encouraging hair regrowth and preventing or halting the progression of the disease, but hairs will not regrow from permanently damaged hair follicles. Although there are no clear guidelines for the management of central centrifugal cicatricial alopecia, and most treatment options are only empiric, but it is imperative to start it early as it would reduce the chance of disease progression.[14]

The response of CCCA to various treatment modalities is slow. Anti-inflammatory therapy is often considered as the first-line treatment and is achieved with the use of topical steroids or intralesional triamcinolone acetonide. Lower concentration of topical steroids decreases the risk of hypopigmentation in people with dark skin. Antibiotics, both topical and systemic like doxycycline, are effective and need to be continued till improvement is seen (at least 2 to 6 months). The dose may be decreased and then gradually discontinued after a quiescent state for at least one year. Systemic anti-inflammatory treatments like mycophenolate mofetil, hydroxychloroquine, and cyclosporine have also been used in a few cases. Short courses of oral corticosteroids are ideal for cases with active inflammation. Vitamin D also has some effective role in management.[2]

Minimal hair grooming is recommended, even though the evidence is not sufficient. Haircare practices need to be modified, reduced or apply for a minimum time. However, excessive traction of hairs should be avoided. Symptomatic relief is achieved with the once-weekly use of mild shampooing.[3]

Central centrifugal cicatricial alopecia clinically resembles:

• Female pattern hair loss: It is a form of non-scarring alopecia occurring after menopause or at puberty, associated with hyperandrogenism. The key feature to differentiate it from CCCA is the absence of scarring and the presence of visible follicular openings.[15]
• Lichen planopilaris: It is a type of scarring alopecia, which is also indistinguishable in some cases. In contrast to central centrifugal cicatricial alopecia, lichen planopilaris presents with perifollicular erythema and follicular keratosis. Frontal fibrosing alopecia is another form of lichen planopilaris. It is characterized by facial papules and slowly progressive scarring alopecia of the scalp. It also affects the eyelashes, eyebrows, and other body parts.[16]
• Tinea capitis: It is a fungal infective condition of the scalp and is differentiated by Wood's lamp examination, which emits bright green fluorescence with Microsporum and faint blue fluorescence with Trichophyton schoenleinii species. It presents both scarring and non-scarring alopecia.
• Discoid lupus erythematosus: It is a form of scarring alopecia that usually affects the scalp. It appears as erythematous scaly plaques with follicular plugging along with pigmentary changes. The histological findings differentiate it from CCCA. Histopathology shows perivascular and peri adnexal lymphohistiocytic infiltrate and interface dermatitis.[16] The basal layer shows degenerative changes.
• Pseudopelade of Brocq: It usually affects middle-aged and older women. It commonly presents as irregular patches of hair loss that usually begins at the vertex. The bald areas look like 'footprints in the snow.' Histopathology of the lesion shows thin epidermis with sclerotic dermis and streamers of fibrosis that goes up to the fat layer.[17]

A photographic scale has been in use for staging alopecia in African-American women. The central scalp alopecia scale used for African-American women helps assess the severity of the hair loss in central centrifugal cicatricial alopecia. It is graded on a scale of 0 to 5. The grade 0 suggests normal hair density and grade 5 suggestive of severe alopecia.[18]

The prognosis of central centrifugal cicatricial alopecia depends on the stage of the disease. Early diagnosis and standard treatment may be able to enhance hair regrowth.[19] The prognosis is not good in patients who present with more advanced disease, because late diagnosis and treatment may only prevent the progression of the disease as there is only a slight potential for hair regrowth due to scarring.[1]

The most common problems associated with central centrifugal cicatricial alopecia relate to late or misdiagnosis. It is a type of scarring alopecia, and thus it is difficult to restore the amount of hair already damaged. Symptoms usually vary from none to very disruptive and presenting with burning, itching, and tenderness. The aim of treatment is to preserve the remaining hairs and avoid the progression of the disease. It is crucial that the patient is clearly explained the severity of the condition and the available treatment options.

• Patient education is essential in allaying anxiety associated with central centrifugal cicatricial alopecia, and well-informed patients can learn to be confident at managing their condition.
• For the scarring stage, it requires emphasizing to the patient that there is no cure, but it can be well controlled and managed primarily.
• It is important that patients should avoid hair practices that need heat treatment or that provide friction.
• The regular use of hair relaxers should be avoided, as it leads to increased disease incidence.

• Consider the impact of CCCA on psychosocial functioning and quality-of-life and remember that it may accompany psychological distress.[20]
• The dermatologist and pharmacist can help to promote the appropriate use of topical corticosteroids and employ steroid-sparing alternatives.
• Hair transplantation can be an effective strategy in patients with advanced disease, although there is a chance that scarring may reduce the survival rate of transplanted graft.
• Patients with advanced disease need to be counseled about various camouflage techniques and hairpieces. These patients always need psychological support and may be encouraged to join various disease support groups.