Central centrifugal cicatricial alopecia: Difference between revisions

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{{Infobox medical condition (new)
 
| name            = <!--{{PAGENAME}} by default-->
{{Infobox medical condition
| synonym        = '''Hot comb alopecia''' and '''Follicular degeneration syndrome'''
| name            = Central centrifugal cicatricial alopecia
| image           =  
| synonyms        = CCCA
| image_size      =
| field           = [[Dermatology]]
| alt            =
| symptoms        = [[Hair loss]], [[scarring]]
| caption        =
| complications  = [[Permanent hair loss]]
| pronounce      =
| onset          = Gradual
| specialty      = dermatology
| duration        = Long-term
| symptoms        =
| causes          = Unknown, possibly [[genetic]]
| complications  =
| risks          = More common in [[African American]] women
| onset          =
| diagnosis      = [[Clinical diagnosis]], [[scalp biopsy]]
| duration        =
| differential    = [[Alopecia areata]], [[Lichen planopilaris]], [[Discoid lupus erythematosus]]
| types          =
| treatment      = [[Topical corticosteroids]], [[antibiotics]], [[antifungal]] medications
| causes          =
| prognosis      = Variable, can lead to permanent hair loss
| risks          =
| frequency      = Common among African American women
| diagnosis      =
| differential    =
| prevention      =
| treatment      =
| medication      =
| prognosis      =
| frequency      =
| deaths          =
}}
}}
'''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.<ref name="skinaging"/> 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]]s, tight braids, heavy extensions, certain oils, gels or pomades.
== Central Centrifugal Cicatricial Alopecia ==
 
'''Central centrifugal cicatricial alopecia''' (CCCA) is a type of [[scarring alopecia]] that primarily affects women of African descent. It is characterized by progressive hair loss that starts at the crown of the scalp and spreads outward in a centrifugal pattern. This condition leads to permanent hair loss due to the destruction of hair follicles and replacement with scar tissue.
==Presentation==
== Pathophysiology ==
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.{{citation needed|date=April 2017}}
The exact cause of CCCA is not fully understood, but it is believed to be multifactorial. Genetic predisposition, [[inflammation]], and [[trauma]] to the hair follicles are considered contributing factors. The condition is associated with the use of certain hair care practices, such as the application of chemical relaxers, heat styling, and tight hairstyles that cause traction on the hair.
 
Histologically, CCCA is characterized by the presence of inflammatory cells around the hair follicles, leading to their destruction and subsequent scarring. The inflammation is primarily lymphocytic, and over time, the affected areas of the scalp show a reduction in the number of hair follicles and an increase in fibrous tissue.
==Cause==
== Clinical Presentation ==
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]]. However, this theory does not ring true as many patients lack a report of traction hairstyling.
Patients with CCCA typically present with hair loss at the vertex of the scalp, which gradually expands in a centrifugal pattern. The hair loss is often accompanied by symptoms such as itching, tenderness, and a burning sensation in the affected area. The scalp may appear shiny due to the presence of scar tissue.
 
The condition is most commonly seen in middle-aged women of African descent, but it can also occur in men and individuals of other ethnicities. The progression of hair loss can vary, with some patients experiencing rapid progression and others having a more indolent course.
== Histopathologic features ==
== Diagnosis ==
Histopathologic features include a perifollicular [[lymphocyte|lymphocytic]] infiltrate, concentric lamellar [[fibrosis]] (layers of [[fibroblast]]s 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<ref>Sperling and Sau, 1992</ref>. Perifollicular [[erythema]] and [[follicular keratosis]] is usually absent.<ref name="skinaging"/>
The diagnosis of CCCA is primarily clinical, based on the characteristic pattern of hair loss and patient history. A scalp biopsy may be performed to confirm the diagnosis and rule out other causes of scarring alopecia. The biopsy typically shows perifollicular fibrosis and a reduction in the number of hair follicles.
 
== Management ==
== Treatment ==
The management of CCCA involves halting the progression of hair loss and alleviating symptoms. Treatment options include:
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.<ref name="skinaging">Woolery-lloyd, Heather. ''Central Centrigugal Scarring Alopecia.'' www.Skinandaging.com, volume 11. (2003)</ref><ref name="Fitz2">Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. {{ISBN|0-07-138076-0}}.</ref>{{rp|648–9}}<ref name="Andrews">James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. {{ISBN|0-7216-2921-0}}.</ref>{{rp|760}}<ref name="jid">Female Pattern Hair Loss and its Relationship to Permanent/Cicatricial Alopecia: A New Perspective. ''Journal of Investigative Dermatology'' (2007) '''127''', 1827-1828</ref>
* Topical and intralesional [[corticosteroids]] to reduce inflammation.
== Epidemiology ==
* [[Antibiotics]] such as doxycycline, which have anti-inflammatory properties.
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.<ref name="skinaging"/>
* [[Hydroxychloroquine]], an antimalarial drug with anti-inflammatory effects.
 
* Avoidance of hair care practices that may exacerbate the condition, such as chemical relaxers and tight hairstyles.
==Terminology==
In advanced cases, surgical options such as hair transplantation may be considered, although the success of such procedures can be limited by the extent of scarring.
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:<ref>Ross EK, Tan E, Shapiro J. J Am Acad Dermatol. 2005 Jul;53(1):1-37;</ref><ref>{{cite web |url=http://www.nahrs.org/home/Default.aspx?tabid%3D63 |title=Archived copy |accessdate=2010-06-24 |url-status=dead |archiveurl=https://web.archive.org/web/20100806195922/http://nahrs.org/home/Default.aspx?tabid=63 |archivedate=2010-08-06 }}</ref>
== Prognosis ==
 
The prognosis of CCCA varies depending on the stage at which treatment is initiated. Early intervention can help prevent further hair loss and manage symptoms, but once scarring has occurred, the hair loss is permanent. Regular follow-up with a dermatologist is recommended to monitor the condition and adjust treatment as needed.
*Hot comb alopecia
== Related Pages ==
*Follicular degeneration syndrome
* [[Alopecia]]
*Pseudopelade in African Americans
* [[Scarring alopecia]]
*Central elliptical pseudopelade in Caucasians
* [[Traction alopecia]]
 
* [[Lichen planopilaris]]
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.<ref>Androgenic pattern presentation of scarring and inflammatory alopecia. J Eur Acad Dermatol Venereol. 2010 Jan 6. Rashid RM, Thomas V.</ref>
[[Category:Dermatology]]
 
[[Category:Hair diseases]]
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.&nbsp;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.
[[Category:Scarring alopecia]]
 
==See also==
* [[Cicatricial alopecia]]
* [[List of cutaneous conditions]]
* [[Hot comb]]
 
==References==
{{reflist}}
 
[[Category:Conditions of the skin appendages]]
[[Category:Syndromes]]
{{dictionary-stub1}}
{{No image}}
__NOINDEX__

Latest revision as of 00:17, 4 April 2025


Central centrifugal cicatricial alopecia
Synonyms CCCA
Pronounce N/A
Specialty N/A
Symptoms Hair loss, scarring
Complications Permanent hair loss
Onset Gradual
Duration Long-term
Types N/A
Causes Unknown, possibly genetic
Risks More common in African American women
Diagnosis Clinical diagnosis, scalp biopsy
Differential diagnosis Alopecia areata, Lichen planopilaris, Discoid lupus erythematosus
Prevention N/A
Treatment Topical corticosteroids, antibiotics, antifungal medications
Medication N/A
Prognosis Variable, can lead to permanent hair loss
Frequency Common among African American women
Deaths N/A


Central Centrifugal Cicatricial Alopecia[edit]

Central centrifugal cicatricial alopecia (CCCA) is a type of scarring alopecia that primarily affects women of African descent. It is characterized by progressive hair loss that starts at the crown of the scalp and spreads outward in a centrifugal pattern. This condition leads to permanent hair loss due to the destruction of hair follicles and replacement with scar tissue.

Pathophysiology[edit]

The exact cause of CCCA is not fully understood, but it is believed to be multifactorial. Genetic predisposition, inflammation, and trauma to the hair follicles are considered contributing factors. The condition is associated with the use of certain hair care practices, such as the application of chemical relaxers, heat styling, and tight hairstyles that cause traction on the hair. Histologically, CCCA is characterized by the presence of inflammatory cells around the hair follicles, leading to their destruction and subsequent scarring. The inflammation is primarily lymphocytic, and over time, the affected areas of the scalp show a reduction in the number of hair follicles and an increase in fibrous tissue.

Clinical Presentation[edit]

Patients with CCCA typically present with hair loss at the vertex of the scalp, which gradually expands in a centrifugal pattern. The hair loss is often accompanied by symptoms such as itching, tenderness, and a burning sensation in the affected area. The scalp may appear shiny due to the presence of scar tissue. The condition is most commonly seen in middle-aged women of African descent, but it can also occur in men and individuals of other ethnicities. The progression of hair loss can vary, with some patients experiencing rapid progression and others having a more indolent course.

Diagnosis[edit]

The diagnosis of CCCA is primarily clinical, based on the characteristic pattern of hair loss and patient history. A scalp biopsy may be performed to confirm the diagnosis and rule out other causes of scarring alopecia. The biopsy typically shows perifollicular fibrosis and a reduction in the number of hair follicles.

Management[edit]

The management of CCCA involves halting the progression of hair loss and alleviating symptoms. Treatment options include:

  • Topical and intralesional corticosteroids to reduce inflammation.
  • Antibiotics such as doxycycline, which have anti-inflammatory properties.
  • Hydroxychloroquine, an antimalarial drug with anti-inflammatory effects.
  • Avoidance of hair care practices that may exacerbate the condition, such as chemical relaxers and tight hairstyles.

In advanced cases, surgical options such as hair transplantation may be considered, although the success of such procedures can be limited by the extent of scarring.

Prognosis[edit]

The prognosis of CCCA varies depending on the stage at which treatment is initiated. Early intervention can help prevent further hair loss and manage symptoms, but once scarring has occurred, the hair loss is permanent. Regular follow-up with a dermatologist is recommended to monitor the condition and adjust treatment as needed.

Related Pages[edit]