Central Centrifugal Cicatricial Alopecia (CCCA) is a type of scarring alopecia that causes permanent hair loss, it affects middle-aged black women the most. It can be observed in men and people of various racial and hair color backgrounds.

Causes:

The cause of CCCA is idiopathic, but most likely multifactorial is the precise cause of CCCA. It has been proposed that there is a genetic component, linked to mutations in the gene PADI3, which codes for peptidyl arginine deiminase, type III (PADI3), an enzyme that alters proteins necessary for the development of the hair shaft. Although hair care procedures including using a hot comb, and tight extensions have been linked for decades, research has not consistently demonstrated this connection. Infections with fungi, bacteria, autoimmune diseases, and heredity disorders are some other variables that have been suggested as causes.

Clinical features:

Usually, hair loss starts at the vertex or middle of the scalp and moves outward in a centrifugal motion. There is loss of the follicular openings on examination of the scalp. As a result, the scalp could seem shiny. Tenderness, itching, and burning are frequent, while some people do not experience any symptoms. Breakage of the hair may potentially be a precursor to CCCA. The loss of hair happens gradually. A photographic rating system has been devised to assess the degree of hair loss in the central region.

Diagnosis:

Early detection of CCCA is crucial because treatment can stop the condition’s progression, frequently leading to significant, irreversible hair loss. Clinical characteristics, a scalp biopsy, and ruling out other hair loss conditions are used to make the diagnosis.

Rather than the middle of a scarred region, a scalp biopsy should be collected from the active edge of an alopecia patch. Histopathology indicates fibrosis and a lymphocytic inflammatory infiltrate surrounding the infundibulum, the root of the hair follicle. One typical trait is premature desquamation, or pealing, of the inner root sheath.

Differential diagnosis:

Any disorder that might cause progressive alopecia that affects the crown can mimic CCCA. These include lichen planopilaris, discoid lupus erythematosus, and even male and female pattern alopecia. True bacterial scalp infections and tinea capitis are additional entities in the differential diagnosis.

Management:

Therapy aims to stop the disease’s progression and stop more hair loss. Regrowth is not possible in locations where fibrosis has replaced the hair follicle. There is no approved targeted therapy for CCCA because the precise etiology is unknown. We can divide it as follows:

Gentle hair care:

Minimizing heat application.
Avoiding tight bands/extensions.
Avoiding tight bands/extensions.
Avoiding hairstyle practices associated with discomfort, and scalp irritation.

Topical medications:

Corticosteroids.
Topical minoxidil.

Intralesional therapy:

Triamcinolone.

Oral therapy:

Doxycycline 100 mg daily for at least 6 months.
Hydroxychloroquine 200 mg BID (or 5 mg/kg/d).

Surgical therapy:

Hair transplantation, once inflammation has been controlled for at least 1 year.

Written by:

Mohammed Alahmadi, Medical Student.

Revised by:

Maee Barakeh, Medical Student.

References:
DermNet
Practical Guide to Dermatology

The Henry Ford Manual
British Association of Dermatologists