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Are You Confident of the Diagnosis?
Central centrifugal cicatricial alopecia (CCCA) is presently considered an idiopathic scarring alopecia of the vertex of the scalp appearing most commonly in African-American women. It presents usually in the third or later decades, average age of onset at 38, as a patch of diminished density of hair on the posterior vertex of the scalp (Figure 1) that continues to widen irregularly but bilaterally, leaving single hairs and islands of hairs in an enlarging sea of shiny, usually normally pigmented skin devoid of follicular orifices. The individual hairs and islands are not elevated above the sea.
Figure 1.
Central centrifugal cicatricial alopecia on the vertex of the scalp.
Characteristic findings on physical examination
Papules are distinctly unusual and pustules absent. Fine scale or erythema are not characteristic though seborrheic dermatitis may easily and commonly accompany this picture. Tingling and tenderness have been described,especially in those patients, initially described as “hot comb alopecia,” but pain and itching are not common complaints. Hair loss elewhere on the body has not been described, nor have cutaneous exanthems.
Expected results of diagnostic studies
Serologic and immunofluorescent testing may be useful to rule out other scarrng alopecias, such as cutaneous lupus or lichen planopilaris, but there are no specific positive findings in CCCA. The histolopathology alone in biopsies showing active inflammation rather than late nonspecific pseudopelade- type scarring is sufficient to rule out these other conditions (Figure 2). Biopsies should be performed on islands or on the periphery of the alopecia, but the usual lack of clinically evident perifollicular inflammation may hinder finding a “best” biopsy site. Biopsy of the midst of scarring will show only nonspecific fibrosis and absence of hair follicles.
Figure 2.
CCCA. Horizontal biopsy. (Courtesy of Bryan Anderson, MD)
Diagnosis confirmation
Other cicatrizing conditions must be ruled out, particularly infectious and measurably treatable conditions. Both Favus and kerion have dramatic inflammatory characteristics not shared by CCCA but late evolved stages may show significant scarring and little residue of the initial character. Cultures should be performed of any scaly areas, scutula and hairs and, for kerion, culture of a hair brush may be more revealing than the inflammatory nodules of the scalp. In classic CCCA unlike folliculitis decalvans, follicular pustules are not seen but any pustulation should be cultured for the possible contribution of staphylococcus aureus or even superimposed tinea capitis.
Idiopathic or autoimmune diseases such as lichen planopilaris and cutaneous lupus have characteristic papules, scale, and follicular erythema on the scalp, and often cutaneous lesions elsewhere that aid in differentiation.
Who is at Risk for Developing this Disease?
Since the cause is unknown the population-at-risk is not clear, but the overwhelming prevalence of CCCA in African-American women continues to suggest that some hair-care practice, be it hot combing, chemical hair relaxation, use of cornrows, or hair weaving or extensions or use of oily hair care products is a trigger factor. Since hair manipulation in women is almost universal statistical studies have not yet pointed to any specific cause, but its consideration is paramount.
What is the Cause of the Disease?
Etiology
Though the disease is overwhelmingly one of females of African ancestry, it was thought to be a behavior-induced process initially from hair management practices. Hot combing allows heated oil to run down the shaft of the stretched vertically-positioned hair and scald the scalp, loosening hairs and initiating the inflammatory response that eventually destroyed the follicle.
Chemical-relaxer-adherent theorists believe that similar scalding occurs from the relaxers and subsequent heat applied in salons and that the vertex is either more sensitive or has more intense exposure to the scalding elements. Traction may be intense, though infrequently repeated in use of cornrowing and hairweaves, but these practices are from a much more modern era than the first appearance of CCCA.
Pathophysiology
The initial response is thought to be premature desquamation of the inner root sheath below the level of the eccrine coil/arrector pili, though this has now been described in at least equal frequency in a variety of other scarring alopecias.
Instead of disappearing at the mid to upper isthmus the inner root sheath disappears in the lower isthmus, allegedly even occasionally below the dermosubcutaneous junction; Sperling hypothesizes that the follicular wall is thus weakened and thins and allows the hair shaft to push through, causing a lymphocytic perifollicular inflammatory response and follicular destruction. For remaining follicles, there is concentric lamellar fibrosis and loss of sebaceous glands; a granulomatous foreign body reaction may ensue if the hair shaft penetrates the follicular wall.
Systemic Implications and Complications
Fortunately, this condition is localized and has no systemic complications other than anxiety and depression, which may require use of a masking non-woven hairpiece for solace.
Treatment Options
Therapeutic choices are limited and anecdotal since the cause is unknown. Treatments have been aimed at using anti-inflammatory medications (topical steroids – triamcinalone 0.1% lotion, spray, cream or ointment, betamethasone valerate or diproprionate or clobetasol solution and antibiotics (doxycycline or minocycline 100mg orally twice daily which have antiinflammatory qualities as well), or tetracycline 500mg orally twice daily, The choice of vehicle is dependent on patient preference.
Cessation of hair manipulation is critical though not substantiated in the literature as the trigger.
Where to apply medications in view of no visible activity and how to judge improvement when there is lack of symptomatology except perhaps from concurrent seborrheic dermatitis suggest a quixotic medical paradigm.
Only repeated photographs and measurement will indicate remission by therapy and no therapy will replenish destroyed follicles. Hair transplantation is unreasonable unless there is real documentation of stable burned-out disease.
Optimal Therapeutic Approach for this Disease
The best approach is to first provide the patient with a realistic assessment that there is no definitive therapy for remission, since we are blind to the cause. Since there is such a clearcut separation by sex in prevalence and in types of hair manipulation by sex it is advisable, despite no statistical model yet able to show the relevance of hair manipulation, that all manipulation be stopped.
Ceasing oily hair care products may not be as relevant here as in folliculitis decalvans or folliculitis papillaris (acne keloid) especially since use of oils in men ,who do not get CCCA except rarely, is as high as in women. Straightening of the hair by heat or by chemicals should be permanently terminated. If the bare area is substantial a hairweave should not be used but a covering atraumatic hairpiece should to provide more social comfort.
Since seborrheic dermatitis is often co-existent efforts to use antiseborrheic shampoos and with frequency greater than weekly, along with prn topical corticosteroid solutions should be encouraged.
Patients should be seen at 3 months after initial treatment is initiated, and then every 6-12 months.
Photographic documentation is helpful to show patients objective information as to whether the disease is improving or progressing.
Unusual Clinical Scenarios to Consider in Patient Management
Persistent pruritus should lead to a search for tinea capitis.
What is the Evidence?
Kyei, A, Bergfeld, W, Piliang, M. “Medical and environmental risk factors for the development of central centrifugal alopecia: a population study”. Arch Dermatol. 2011 Apr11. (Population-based study that attempts to determine various risk factors for the development of CCCA.)
Shah, S, Alexis, A. “Central centrifugal cicatricial alopecia: retrospective chart review”. J Cut Med Surg. vol. 14. 2010. pp. 212-22. (Large retrospective review of patients that were classified with CCCA. Discusses various characteristics seen.)
Khumalo, N. “Grooming and central centrifugal alopecia”. J Am Acad Dermatol. vol. 62. 2010. pp. 507-8. (Article on CCCA and the affects of grooming.)
Fu, J, Price, V. “Approach to hair loss in women of color”. Semin Cut Med Surg. vol. 28. 2009. pp. 109-14. (Excellent practical clinical approach to the female patient with hair loss.)
Gathers, R, Lim, H. “Central centrifugal cicatricial alopecia: past, present and future”. J Am Acad Dermatol. vol. 609. 2009. pp. 660-68.
Olsen, E, Callender, V, Sperling, L. “Central scalp alopecia photographic scale in African-American women”. Dermatol Ther. vol. 21. 2008. pp. 264-7. (Novel CCCA scale for following and documenting clinical course.)
Sperling, L. “Scarring alopecia and the dermatopathologist”. J Cutan Pathol. vol. 28. 2001. pp. 333-42. (Pathological review of scarring alopecias and findings seen histologically.)
Sperling, L, Sau, P. “The follicular degeneration syndrome in black patients”. Arch Dermatol. vol. 128. 1992. pp. 68-74. (Discussion of the follicular degeneration syndrome, defines the syndrome.)
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