Central centrifugal cicatricial alopecia (CCCA), is a type of

alopecia Hair loss, also known as alopecia or baldness, refers to a loss of hair from part of the head or body. Typically at least the head is involved. The severity of hair loss can vary from a small area to the entire body. Inflammation or scarri ...

first noticed in

African Americans African Americans (also referred to as Black Americans and Afro-Americans) are an ethnic group consisting of Americans with partial or total ancestry from sub-Saharan Africa. The term "African American" generally denotes descendants of ens ...

in the 1950s and reported by LoPresti et al. in 1968 as a result of application of

petrolatum Petroleum jelly, petrolatum, white petrolatum, soft paraffin, or multi-hydrocarbon, CAS number 8009-03-8, is a semi-solid mixture of hydrocarbons (with carbon numbers mainly higher than 25), originally promoted as a topical ointment for its he ...

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 A relaxer is a type of lotion or cream generally used by people with tight curls or very curly hair which makes hair easier to straighten by chemically "relaxing" the natural curls. The active agent is usually a strong alkali, although some formu ...

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

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 The Hamilton–Norwood scale is used to classify the stages of male pattern baldness. The stages are described with a number from 1 to 7. Overview This measurement Measurement is the quantification of attributes of an object or event, ...

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

Cause

The mechanism of pathology of CCCA remains unknown; thus, the cause has only been postulated and not proven. CCCA is suspected to have a multi-factored cause. However, one theory involves pressure exerted on the internal root sheath leading to damage, which leads to the recruitment of inflammatory cells and the result of scarring. African Americans are found to be at increased risk. Historically, some have hypothesized that CCCA represents an end stage of

traction alopecia Traction alopecia is a type of alopecia or hair loss caused by a chronic pulling force being applied to the hair. It commonly results from a person frequently wearing their hair in a particularly tight ponytail, pigtails, or braids with increased o ...

. However, the veracity of this theory is low as many patients who have CCCA have not employed traction hairstyling.

Histopathologic features

Histopathologic features include a perifollicular

lymphocytic A lymphocyte is a type of white blood cell (leukocyte) in the immune system of gnathostomata, most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated immunity, cell-mediated, cytotoxicity, cytotoxic innate imm ...

infiltrate, concentric lamellar

fibrosis Fibrosis, also known as fibrotic scarring, is a pathological wound healing in which connective tissue replaces normal parenchymal tissue to the extent that it goes unchecked, leading to considerable tissue remodelling and the formation of perma ...

(layers of

fibroblast A fibroblast is a type of cell (biology), biological cell that synthesizes the extracellular matrix and collagen, produces the structural framework (Stroma (tissue), stroma) for animal Tissue (biology), tissues, and plays a critical role in wound ...

s in the papillary dermis),

sebaceous gland A sebaceous gland is a microscopic exocrine gland in the skin that opens into a hair follicle to secrete an oily or waxy matter, called sebum, which lubricates the hair and skin of mammals. In humans, sebaceous glands occur in the greatest number ...

loss and premature disintegration of the internal root sheath. Additionally,

granulomatous inflammation A granuloma is an aggregation of macrophages that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate. Such substances include infectious ...

secondary to follicular rupture has been noted. Perifollicular

erythema Erythema (from the Greek , meaning red) is redness of the skin or mucous membranes, caused by hyperemia (increased blood flow) in superficial capillaries. It occurs with any skin injury, infection, or inflammation. Examples of erythema not assoc ...

and

follicular keratosis A seborrheic keratosis is a non-cancerous (benign) skin tumour that originates from cells, namely keratinocytes, in the outer layer of the skin called the epidermis. Like liver spots, seborrheic keratoses are seen more often as people age. The tu ...

is usually absent.

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, sold under the brand name Prograf among others, is an immunosuppressive drug. After allogeneic organ transplant, the risk of organ rejection is moderate. To lower the risk of organ rejection, tacrolimus is given. The drug can also ...

(Protopic) and

pimecrolimus Pimecrolimus is an immunomodulating agent of the calcineurin inhibitor class used in the treatment of atopic dermatitis (eczema). It is available as a topical cream, once marketed by Novartis (however, Galderma has been promoting the compound in ...

(Elidel), and anti-androgen/5alpha Reductase inhibitors have been used with unknown efficacy.Woolery-lloyd, Heather. ''Central Centrigugal Scarring Alopecia.'' www.Skinandaging.com, volume 11. (2003)Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. .James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. .Female Pattern Hair Loss and its Relationship to Permanent/Cicatricial Alopecia: A New Perspective. ''Journal of Investigative Dermatology'' (2007) 127, 1827-1828

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.

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 North is one of the four compass points or cardinal directions. It is the opposite of south and is perpendicular to east and west. ''North'' is a noun, adjective, or adverb indicating direction or geography. Etymology The word ''north'' is ...

. 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 In botany, apomixis is asexual reproduction without fertilization. Its etymology is Greek for "away from" + "mixing". This definition notably does not mention meiosis. Thus "normal asexual reproduction" of plants, such as propagation from cutt ...

. Alopecia mimics have proven a problem in establishing diagnosis of alopecia when using only clinical evaluation.Androgenic pattern presentation of scarring and inflammatory alopecia. J Eur Acad Dermatol Venereol. 2010 Jan 6. Rashid RM, Thomas V. 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.

References

{{reflist Conditions of the skin appendages Syndromes