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Central centrifugal cicatricial alopecia introduction
The cause and pathogenesis of unprecedented hair loss due to alopecia
is still largely sheathed in mystery. Limited by the knowledge and
information available today, dermatologists need to sift through
the diagnostic hallmarks of these anomalies to advise alopecia patients
on appropriate therapeutic measures. The stumbling block is that
many of the features of each type of alopecia overlap and a conclusive
diagnosis is often elusive. Researchers and authorities in the field
of hair loss have endeavored to provide a classification of alopecias
on the basis of pathology, which gives clinicians a diagnostic and
investigational framework to guide them in their identification
and analysis.
Central centrifugal cicatricial alopecia (CCCA) is one such diagnostic
category adopted by the North American Hair Research Society consensus
group to encompass the terms hot comb alopecia, follicular degeneration
syndrome, pseudopelade in African Americans and central elliptical
pseudopelade in Caucasians. These conditions seem to be clinical
subsets of the same pathological process with certain common characteristics.
Common clinical features include circle shaped balding, principally
on the crown or the vertex of the scalp, histological evidence of
inflammation in the area of disease activity, chronic and rapidly
progressive cutaneous changes with eventual “burnout” of
the disease.
In general, central centrifugal cicatricial alopecia is a condition
that presents flesh colored, overtly non inflammatory (i.e. you
can’t see any inflammation just by looking at the skin) cicatricial
alopecia of the central scalp, which enlarges centrifugally as the
disease progresses. This group of rare disorders completely destroys
the hair follicle and replaces it with scar tissue, causing permanent
hair loss. The condition is not to be confused with central centrifuging
scarring alopecia, as defined by Sperling et al, and both conditions
indicate different clinical conceptions.
Central centrifugal cicatricial alopecia was originally called “hot-comb
alopecia”. According to a study done on 51 African American
women, heat from hot combing or use of oil pomades for hair straightening
was thought to be the factor responsible for this condition. Patients
complained of soreness of the scalp during or immediately after
the procedure. The spreading alopecia of the central part of the
scalp, thought to result from the repeated use of the procedure,
commonly exhibited clusters of 4-7 hairs which emerged from dilated
sunken openings filled with debris. Subsequently it became evident
that usage of the hot comb was not the sole cause of this pattern
of hair loss, and hot comb alopecia was renamed follicular degeneration
syndrome.
Central centrifugal cicatricial alopecia is perhaps the most common
as well as the most challenging form of alopecia seen in the United
States. It affects black women predominantly, but African American
men can also be affected. It remains to be ascertained whether
central centrifugal cicatricial alopecia is a unique classifiable
entity on its own or merely a morphological pattern shared by
some distinctly different alopecias. There may be a hereditary
component to the condition and for some Black women, a combination
of hair care practices might contribute to further aggravate the
condition.
Central
centrifugal cicatricial alopecia clinical
features
The term central centrifugal cicatricial alopecia coined by NAHRS
now stands for follicular degeneration syndrome, which typically
affects adult black women and occasionally men. Documentation by
Sperling and Sau describe the symptoms of central centrifugal cicatricial
alopecia as a ‘pins and needles’ sensation accompanied
by pruritis and tenderness. Slowly progressive, symmetric centrifugal
scarring takes place on the vertex of the scalp without obvious
inflammation. The scarred skin is usually supple, shiny and flesh
colored. Patches of unaffected hair may be in existence within areas
of the scar. Once the hair follicles become damaged or destroyed
and scar tissue forms, the hair does not re-grow.
Perifollicular hyper pigmentation and polytrichia (excessive hairiness)
may or may not be observed. In men, central centrifugal cicatricial
alopecia syndrome is accompanied by perifollicular, firm, and inflammatory
papules within the areas of hair loss.
Central
centrifugal cicatricial alopecia differential diagnosis
The forms of primary scarring alopecia present problems in diagnosis
for both the clinician as well as the pathologist. Features are
very often common to more than one form, and the greater is the
overlap, the greater is the diagnostic dilemma. Central centrifugal
cicatricial alopecia shares some of the same signs and symptoms
as well as pathological features with classic Pseudopelade of Brocq
(an insidious form of primary alopecia), Androgenetic alopecia (hair
loss mediated by the presence of the androgen dihydrotestosterone)
and trichotillomania, (a compulsive behavior involving the repeated
plucking of one's hair) and must be distinguished from these during
diagnosis.
Central
centrifugal cicatricial alopecia pathology
It is the pathologists who can help differentiate one form of scarring
alopecia from another, and give direction to clinicians arrive at
a definitive diagnosis. To do this, a scalp biopsy is performed
which is a simple procedure in which a small area of the scalp is
removed after numbing medication is administered. The site chosen
for scalp biopsy is crucial to the evaluation’s success, as
the pathogenic information obtained from a hair bearing site with
active disease is more productive than from bald or end-stage diseased
areas of the scalp. Features of the follicular degeneration syndrome
are best demonstrated in transverse sections as transverse sectioning
allows for viewing of follicles at multiple levels.
Premature desquamation of the inner root sheath serves as a histologic
marker for follicular degeneration syndrome, and may be an important
pathogenetic factor. Put simply, it means that the inner root sheath
disappears very low in the hair follicle. In most cases of primary
cicatricial alopecia, histopathologic examination reveals inflammation
that affects the upper part of the hair follicle. In follicular
degeneration syndrome, premature desquamation can also be seen in
the non inflamed follicles as well as in ‘normal’ scalp
tissue. Researchers opine that observation of this feature in the
early stages of the disease and in the absence of inflammation is
a pointer towards follicular degeneration syndrome.
In clinically active disease, there is an evidence of a perifollicular
lymphocytic infiltrate surrounding the upper follicle. There is
epithelial atrophy or thinning which is evident at the level of
the isthmus (the short segment of the hair follicle that extends
from the insertion of the erector pili muscle to the entrance of
the sebaceous gland duct) and the lower infundibulum (the segment
of the hair follicle that extends from the entrance of the sebaceous
gland duct to the follicular orifice). Concentric lamellar fibroplasia
encircles the zone of epithelial thinning. Not all hair follicles
are affected.
In advanced stages of the disorder, perifollicular granulomatous
inflammations in the form of hair-shaft foreign-body giant cells
are present. A granuloma is a mass of inflamed tissue, usually associated
with ulcerated infections. End stage disease is marked by follicular
fibrosis with retention of arrector pili. The arrector pili muscle
is a source of information when evaluating a skin biopsy, as it
is well innervated with autonomic nerves that control when the muscle
contracts. The VVG staining pattern resembles that seen in Pseudopelade
of Brocq and is not a conclusive test for CCCA.
Central
centrifugal cicatricial alopecia treatment
Alopecia is a devastating condition for anyone, irrespective of
the cause. Hair loss effects self-esteem, social interactions and
relationships, and early diagnosis and prevention of disease progress
are crucial to the wellbeing of the patient.
As is the case with most of the primary alopecias, the etiology
of central centrifugal cicatricial alopecia eludes us. It is unclear,
but suspected, that this condition may be related to chemical processing,
heat, or chronic tension on the hair. Although the veracity of this
theory still hangs in the balance, some experts are of the opinion
that harsh hair grooming practices and chemical hair care products
that damage hair cells should not be used by persons showing signs
of central centrifugal cicatricial alopecia. Discontinuation of
harsh styling practices and substituting gentler techniques could
allow the inflammation to subside, the scalp to heal and initiate
the process of hair growth.
The bottom line is that it is the permanent disruption of the critical
elements required for follicular reconstitution that results in
permanent alopecia. The evaluation of persons presenting features
of cicatricial alopecia is a challenging one for dermatologists.
A thorough examination of the patient, evaluation of extra-cranial
cutaneous features and symptoms, and due consideration to pathological
and clinical findings must be made before arriving at a diagnosis
and choosing the line of treatment.
Sperling et al have reported that daily use of a potent topical
corticosteroid in conjunction with tetracycline has been successful
in treating CCCA. Once satisfactory improvement is noticed, the
treatment doses are usually tapered and stopped only when the disease
has been in remission for a full year.
In severe central centrifugal cicatricial alopecia the hair follicles
are completely destroyed and neither topical nor injected medications
can help reverse the condition. Hair transplantation may be a solution
for some women although the severe scarring may make this procedure
technically difficult.
In conclusion, despite the on-going debate as to the
nature of the hair disorder, it has been acknowledged in the dermo-pathological
scenario that
some women do develop an insidious, non-inflammatory primary cicatricial
alopecia of the central scalp that spreads centrifugally. Whether
central centrifugal cicatricial alopecia is caused by an inherited
follicular defect or is the result of exogenous factors such as
the use of harsh hair care products or procedures remains to be
proven.
Central
centrifugal cicatricial alopecia references
- Sperling LC. Scarring
alopecia and the dermatopathologist. J Cutan Pathol. 2001 Aug;28(7):333-42.
PMID: 11437938
- Sperling LC, Skelton HG 3rd, Smith KJ, Sau P,
Friedman K. Follicular degeneration syndrome
in men. Arch Dermatol. 1994 Jun;130(6):763-9. PMID: 8002648
- Sperling LC, Sau P. The follicular degeneration syndrome in
black patients. 'Hot comb alopecia' revisited and revised. Arch
Dermatol. 1992
Jan;128(1):68-74. PMID: 1739290
- LoPresti P, Papa CM, Kligman AM.
Hot comb alopecia. Arch Dermatol. 1968 Sep;98(3):234-8. PMID:
5673883
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