Central centrifugal cicatricial alopecia

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Central centrifugal cicatricial alopecia
Other namesHot comb alopecia and Follicular degeneration syndrome
Specialty Dermatology

Central centrifugal cicatricial alopecia (CCCA), is a type of alopecia 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. [1] 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 relaxers, tight braids, heavy extensions, certain oils, gels or pomades.

Contents

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 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. [2]

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. [2] 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. 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 infiltrate, concentric lamellar fibrosis (layers of fibroblasts in the papillary dermis), sebaceous gland loss and premature disintegration of the internal root sheath. Additionally, granulomatous inflammation secondary to follicular rupture has been noted. [3] Perifollicular erythema and follicular keratosis is usually absent. [1]

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 (Protopic) and pimecrolimus (Elidel), and anti-androgen/5alpha Reductase inhibitors have been used with unknown efficacy. [1] [4] : 648–9  [5] : 760  [6]

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. [1]

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: [7] [8]

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. Alopecia mimics have proven a problem in establishing diagnosis of alopecia when using only clinical evaluation. [9]

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.

See also

Related Research Articles

<span class="mw-page-title-main">Hair loss</span> Loss of hair from the head or body

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 scarring is not usually present. Hair loss in some people causes psychological distress.

Traction alopecia is a type of hair loss caused by a pulling force being applied to the hair.

<span class="mw-page-title-main">Hot comb</span> Hair straightening tool

A hot comb is a metal comb that is used to straighten moderate or coarse hair and create a smoother hair texture. A hot comb is heated and used to straighten the hair from the roots. It can be placed directly on the source of heat or it may be electrically heated.

The management of hair loss, includes prevention and treatment of alopecia, baldness, and hair thinning, and regrowth of hair.

<span class="mw-page-title-main">Pattern hair loss</span> Medical condition

Pattern hair loss is a hair loss condition that primarily affects the top and front of the scalp. In male-pattern hair loss (MPHL), the hair loss typically presents itself as either a receding front hairline, loss of hair on the crown (vertex) of the scalp, or a combination of both. Female-pattern hair loss (FPHL) typically presents as a diffuse thinning of the hair across the entire scalp.

Uncombable hair syndrome Rare scalp hair shaft dysplasia

Uncombable hair syndrome (UHS) is a rare structural anomaly of the hair with a variable degree of effect. It is characterized by hair that is silvery, dry, frizzy, wiry, and impossible to comb. It was first reported in the early 20th century. It typically becomes apparent between the ages of 3 months and 12 years. UHS has several names, including “pili trianguli et canaliculi,” “cheveux incoiffables,” and “spun-glass hair.” This disorder is believed to be autosomal recessive in most instances, but there are a few documented cases where multiple family members display the trait in an autosomal dominant fashion. Based on the current scientific studies related to the disorder, the three genes that have been causally linked to UHS are PADI3, TGM3, and TCHH. These genes encode proteins important for hair shaft formation. Clinical symptoms of the disorder arise between 3 months and 12 years of age. The quantity of hair on the head does not change, but hair starts to grow more slowly and becomes increasingly “uncombable.” To be clinically apparent, 50% of all scalp hair shafts must be affected by UHS. This syndrome only affects the hair shaft of the scalp and does not influence hair growth in terms of quantity, textural feel, or appearance on the rest of the body.

Anagen effluvium is the pathologic loss of anagen or growth-phase hairs. Classically, it is caused by radiation therapy to the head and systemic chemotherapy, especially with alkylating agents.

Loose anagen syndrome Medical condition

Loose anagen syndrome, also known as loose anagen hair syndrome, is a hair disorder related to dermatology. It is characterised by the easy and pain free detachment of anagen staged hairs from the scalp. This hair condition can be spontaneous or genetically inherited.

Scarring hair loss, also known as cicatricial alopecia, is the loss of hair which is accompanied with scarring. This is in contrast to non scarring hair loss.

<span class="mw-page-title-main">Folliculitis decalvans</span> Medical condition

Folliculitis decalvans is an inflammation of the hair follicle that leads to bogginess or induration of involved parts of the scalp along with pustules, erosions, crusts, ulcers, and scale. It begins at a central point and spreads outward, leaving scarring, sores, and, due to the inflammation, hair loss in its wake. No permanent cure has been found for this condition, but there is promise in a regimen of dual therapy with rifampin 300 mg twice daily and clindamycin 300 mg twice daily. This new treatment can be used to control the condition, and tests have indicated that after 3 to 5 months long uninterrupted courses of treatment, many patients have seen limited to no recurrence.

Acne necrotica presents with a primary lesion that is a pruritic or painful erythematous follicular-based papule that develops central necrosis and crusting and heals with a varioliform scar.

Atrichia with papular lesions is a diffuse hair loss caused by an abnormality of the human homologue of the mouse hairless gene.

Hot comb alopecia was first reported in the late 1960s as a scarring alopecia seen in black women who straightened their hair with hot combs for cosmetic purposes, developing characteristically on the crown and spreading peripherally to form a large oval area of partial hair loss.

Tufted folliculitis presents with doll's hair-like bundling of follicular units, and is seen in a wide range of scarring conditions including chronic staphylococcal infection, chronic lupus erythematosus, lichen planopilaris, Graham-Little syndrome, folliculitis decalvans, acne keloidalis nuchae, immunobullous disorders, and dissecting cellulitis.

Keratosis pilaris atrophicans faciei begins in infancy as follicular papules with perifollicular erythema. Initially, the lesions are restricted to the lateral eyebrows, but with time spread to involve the cheeks and forehead, and may also be associated with keratosis pilaris on the extremities and buttocks.

Tumor alopecia is the hair loss in the immediate vicinity of either benign or malignant tumors of the scalp.

Non scarring hair loss, also known as noncicatricial alopecia is the loss of hair without any scarring being present. There is typically little inflammation and irritation, but hair loss is significant. This is in contrast to scarring hair loss during which hair follicles are replaced with scar tissue as a result of inflammation. Hair loss may be spread throughout the scalp (diffuse) or at certain spots (focal). The loss may be sudden or gradual with accompanying stress.

Trichoscopy

Trichoscopy is a method of hair and scalp evaluation and is used for diagnosing hair and scalp diseases. The method is based on dermoscopy. In trichoscopy hair and scalp structures may be visualized at many-fold magnification. Currently magnifications ranging from 10-fold to 70-fold are most popular in research and clinical practice.

Frontal fibrosing alopecia Medical condition

Frontal fibrosing alopecia is the frontotemporal hairline recession and eyebrow loss in postmenopausal women that is associated with perifollicular erythema, especially along the hairline. It is considered to be a clinical variant of lichen planopilaris.

Ncoza Dlova is a South African dermatologist. In 2019, she helped discover a new gene that is a major cause of permanent hair loss amongst women of African descent. She is currently the dean and the first African woman to head University of KwaZulu-Natal's School of Clinical Medicine.

References

  1. 1 2 3 4 Woolery-lloyd, Heather. Central Centrigugal Scarring Alopecia. www.Skinandaging.com, volume 11. (2003)
  2. 1 2 Wang EH, Monga I, Sallee BN, Chen JC, Abdelaziz AR, Perez-Lorenzo R, Bordone LA, Christiano AM (Jul 2022). "Primary cicatricial alopecias are characterized by dysregulation of shared gene expression pathways". PNAS Nexus. 1 (3): pgac111. doi:10.1093/pnasnexus/pgac111. PMC   9308563 . PMID   35899069.
  3. ↑ Sperling and Sau, 1992
  4. ↑ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN   0-07-138076-0.
  5. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN   0-7216-2921-0.
  6. ↑ Female Pattern Hair Loss and its Relationship to Permanent/Cicatricial Alopecia: A New Perspective. Journal of Investigative Dermatology (2007) 127, 1827-1828
  7. ↑ Ross EK, Tan E, Shapiro J. J Am Acad Dermatol. 2005 Jul;53(1):1-37;
  8. ↑ Dirk M. Elston; Elise Olsen. "Cicatricial Alopecia". North American Hair Research Society. Archived from the original on 2010-08-06. Retrieved 2010-06-24.
  9. ↑ Androgenic pattern presentation of scarring and inflammatory alopecia. J Eur Acad Dermatol Venereol. 2010 Jan 6. Rashid RM, Thomas V.
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