Central centrifugal cicatricial alopecia, hot comb alopecia, or follicular degeneration syndrome is a type of alopecia

Alopecia

Alopecia means loss of hair from the head or body. Alopecia can mean baldness, a term generally reserved for pattern alopecia or androgenic alopecia. Compulsive pulling of hair can also produce hair loss. Hairstyling routines such as tight ponytails or braids may induce Traction alopecia. Both...

 first noticed in African Americans in the 1950s and reported by LoPresti et al. in 1968 as a result of application of petrolatum followed by a stove-heated iron comb. The original theory was that the hot petrolatum would travel down to the hair root, burn the follicle, and after repetitive injury scarring would result. Later CCCA was realized to affect men and women without a history significant for use of such styling techniques. Consequently, the terms "follicular degeneration syndrome" per Sperling and Sau in 1992 and then CCCA per Olsent et al. in 2003 were evolved. Plausible contributing factors may include other African-American styling techniques such as relaxer

Relaxer

A relaxer is a type of lotion or cream generally used by people with afro textured hair, which makes hair less curly and also easier to straighten by chemically "relaxing" the natural curls...

s, tight braids, heavy extensions, certain oils, gels or pomades.

Terminology

The terminology of CCCA has been a source of regular confusion. Recent clarifications have been made, with the term "central centrifugal cicatritial alopecia" adopted as a diagnostic category by the North American Hair Research Society. It has also been referred to as:

• Hot comb alopecia
• Follicular degeneration syndrome
• Pseudopelade in African Americans
• Central elliptical pseudopelade in Caucasians

Also in this category is cicatricial pattern hair loss (CPHL). This CCCA pattern is a potential alopecia mimic that can be confused for androgenetic alopecia

Androgenetic alopecia

Androgenic alopecia is the most common cause of hair loss and thinning in humans. Variants appear in both men and women. Androgenic alopecia also occurs in chimpanzees, and orangutans. In humans, this condition is also commonly known as male pattern baldness...

. Alopecia mimics have proven a problem in establishing diagnosis of alopecia when using only clinical evaluation.

A similarly sounding term is central centrifugal scarring alopecia (CCSA). (L.C. Sperling, Central, centrifugal scarring alopecia. In: L.C. Sperling, Editor, An atlas of hair pathology with clinical correlations, Parthenon Publishing Group, New York (2003), pp. 91–100). This is a clinical finding that describes the diagnosis of some primary cicatricial alopecias as noted mainly in the central scalp, and includes CCCA, folliculitis decalvans, and any other potential centrally presenting cicatricial alopecia. This term is not often used in the literature to signify diagnostic terminology.

Epidemiology

CCCA tends to present itself in the 20s and progresses over 20-30 years. One should consider this diagnosis in African Americans with what appears to be a female-pattern hair loss.

Etiology

The mechanism of pathology of CCCA has only been postulated and not proven. However, one theory involves pressure exerted on the internal root sheath leading to damage, which leads to the recruitment of inflammatory cells and the end result of scarring. African Americans are found to be at increased risk either because of the curled hair shaft, distinct styling practices, moisturizing hair products, or chemical processing techniques (especially in the youth population). Some have hypothesized that CCCA represents an end stage of traction alopecia

Traction alopecia

Traction alopecia is a form of alopecia, or gradual hair loss, caused primarily by pulling force being applied to the hair. This commonly results from the sufferer frequently wearing his/her hair in a particularly tight ponytail, pigtails, or braids...

. However, this theory does not ring true as many patients lack a report of traction hairstyling.

Histopathologic features

Histopathologic features include perifollicular lymphocytic infiltrate, concentric lamellar fibrosis (layers of fibroblast in papillary dermis), sebaceous gland loss and premature disintegration of the internal root sheath. Additionally, granulomatous inflammation secondary to follicular rupture has been noted (Sperling and Sau, 1992). Perifollicular erythema and follicular keratosis is usually absent.

Clinical presentation

CCCA usually begins at the central (sagittal) midline of the scalp. It is symmetric and exhibits scarring as the name suggests. It involves solely the top of the scalp or may progress to Hamilton-Norwood scale

Hamilton-Norwood scale

The progression of male pattern baldness is generally classified on the Hamilton-Norwood scale, which ranges from stages I to VII.This measurement scale was first introduced by Dr. James Hamilton in the 1950s and later revised and updated by Dr. O'Tar Norwood in the 1970s....

 Type VI or VII. Early symptoms may include pruritis, dysesthesias and tenderness. On examination the skin is thin with few follicular ostia and later in the disease the scalp may appear shiny.

Treatment

Treatments for CCCA remain investigational. Altering hair care practices has not been proven to assist in hair rejuvenation. High-dose topical steroids, antibiotics, immunomodulators such as tacrolimus

Tacrolimus

Tacrolimus is an immunosuppressive drug that is mainly used after allogeneic organ transplant to reduce the activity of the patient's immune system and so lower the risk of organ rejection...

 (Protopic) and pimecrolimus

Pimecrolimus

Pimecrolimus is an immunomodulating agent used in the treatment of atopic dermatitis . It is currently available as a topical cream, once marketed by Novartis under the trade name Elidel.-Pharmacology:Pimecrolimus is an ascomycin macrolactam derivative...

(Elidel), and anti-androgen/5alpha Reductase inhibitors have been used with unknown efficacy.