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Frontal
fibrosing alopecia introduction
The evaluation of people presenting features of cicatricial alopecia
is a challenging one for dermatologists. Many of the features of
each type of alopecia overlap and a conclusive diagnosis is often
elusive. Earlier, dermatologists and pathologists had very little
input to assist them in the assessment of hair disease. But today,
dermatologists have a more in-depth knowledge of alopecia, its classification
and the tools that can be used for determining the kind of hair
disease.
Because of these developments in disease diagnosis, relatively
recently a condition in postmenopausal women characterized by
progressive
frontal hairline recession associated with scarring was described
and named as frontal fibrosing alopecia. Frontal fibrosing alopecia
was first described by Kossard in 1994 as a new clinical entity
that could be regarded as a version of lichen planopilaris with “fronto-temporal” and
associated eyebrow localization. Further cases of frontal fibrosing
alopecia have been reported to date, taking into account clinical,
histological and pathological information.
Frontal fibrosing alopecia is also known as postmenopausal frontal
fibrosing alopecia. Female hair loss occurs in more than one pattern,
and it is common knowledge that hair loss has a significant effect
on the social and psychological well-being of the patient. This
particular form of alopecia is considered a variant of lichen
planopilaris in a patterned distribution that mainly affects postmenopausal
women
with a mean age of 67 years. However, there are a few isolated
reports of the condition in pre-menopausal women. The onset of
frontal fibrosing
alopecia can occur any time after menopause, whether the menopause
is natural or surgically triggered. The exact cause of frontal
fibrosing alopecia is unknown. One probable reason might be the
disturbed
immune response to some component of the scalp hair follicles,
however, whether or not the hair loss is caused by hormonal fluctuations
is yet to be ascertained.
Frontal
fibrosing alopecia clinical
features
Post menopausal women over 40 years of age are typically victims
of frontal fibrosing alopecia. The disorder presents as a band-like
anterior alopecia that progressively spreads to the temporal parietal
scalp. Pruritis of the fronto-temporal hairline is commonly found,
and there may be loss of follicular ostia or openings.
The affected area in victims of postmenopausal frontal fibrosing
alopecia appears as a shiny band-like zone of incomplete hair loss,
and the recession of the frontal hairline is a common event. The
new hairline is serrated (jaggy) and it frequently contains hairs
with perifollicular erythema (reddening) and hyperkeratosis (thickening),
and it is difficult to distinguish the condition from lichen planopilaris.
The skin in the affected area, which may be from one to eight centimeters
in width, is usually pale or mildly scarred, and stands out in sharp
contrast to the skin of the forehead. The margin of the band of
alopecia may be marked by follicular erythema or inflammation and
papules. In frontal fibrosing alopecia, the eyebrows are often thinned
and may even be absent. There are also rare cases of associated
eyelash and abdomen hair loss reported.
Frontal fibrosing alopecia or postmenopausal frontal fibrosing
alopecia is often insidious, but can also be rapidly progressive
in certain cases.
Frontal
fibrosing alopecia differential
diagnosis
The ability of the clinician to recognize that the hair loss process
is a scarring or non-scarring one is critical for accurate diagnosis.
The greater is the overlap of clinical and histological features,
the greater is the diagnostic complexity. When histological features
are non-specific, it points to more than one clinical entity.
Frontal fibrosing alopecia or postmenopausal frontal fibrosing
alopecia can be confused with other forms of hair loss like Keratosis
follicularis spinulosa decalvans (KFSD), Graham-Little syndrome
and traction alopecia. Often the condition may also be confused
with androgenetic alopecia (female pattern hair loss). The histological
findings are indistinguishable from those seen in lichen planopilaris.
However, the absence of associated lesions of lichen planus in case
study subjects raises the possibility that this mode of follicular
destruction represents a reaction pattern triggered by the events
underlying postmenopausal frontal hairline recession.
Postmenopausal frontal fibrosing alopecia as a disease must be
differentiated from other forms of fibrosing alopecia including
discoid lupus erythematosus, folliculitis decalvans, keloid acne
and lichen planopilaris. The disease should also be differentiated
from traction alopecias that lead to progressive miniaturization
of the follicles.
Postmenopausal frontal fibrosing alopecia has similarities to lichen
planopilaris but it is distinguishable by a distinctive symmetrical
fronto-temporal distribution and a progressive course. In case studies
of postmenopausal frontal fibrosing alopecia patients, no evidence
of lichen planus was observed at other sites. Non-scarring, apparently
non- inflammatory symmetric hair loss is a characteristic feature
of postmenopausal frontal fibrosing alopecia.
Frontal
fibrosing alopecia pathology
Pathology is that branch of medicine that studies the causes and
nature of diseases, especially the structural and functional changes
brought about by diseases. It is the fundamental task of the pathologist
to help differentiate one form of scarring alopecia from another,
to help clinicians arrive at a definitive diagnosis. Scalp biopsy
from a clinically active area of scalp involvement is always crucial
to the assessment and diagnosis of the patient presenting symptoms
of cicatricial alopecia. As different skin conditions can often
look similar to the naked eye, additional information obtained by
looking at the structure of the skin under the microscope after
the cells have been stained with special colored dyes can help in
reliable diagnosis.
If only one biopsy is taken, it is recommended that it is submitted
for transverse sectioning (cuts across the hair follicles) as compared
to vertical sectioning (cuts down the length of the hair follicles),
as this allows examination of all the follicles at multiple levels,
and offers the ability to quantify the results. Some authors are
in favor of both transverse and vertical sectioning and to do this
the biopsy is cut in half and each half is processed separately.
Routine evaluations when assessing postmenopausal frontal fibrosing
alopecia reveal features that are not easily distinguishable from
classic lichen planopilaris. Scalp biopsy specimens from the frontal
hair margin show perifollicular fibrosis and lymphocytic inflammation
concentrated around the isthmus (the short segment that extends
from the insertion of the erector pili muscle to the entrance of
the sebaceous gland duct) and infundibular areas (the infundibulum
is the segment that extends from the entrance of the sebaceous gland
duct to the follicular orifice of the follicles). Immuno-phenotyping
of the lymphocytes shows a dominance of activated T-helper cells.
Patients under case study have not had evidence of lichen planus
elsewhere.
Frontal
fibrosing alopecia treatment
Frontal fibrosing alopecia is an irreversible process with a slow
course and there is no clearly defined line of treatment for the
condition. Findings of the scalp biopsy, information of the type
of inflammation present, location and amount of scalp changes all
determine the degree of activity and the selection of appropriate
therapy.
Progress of postmenopausal frontal fibrosing alopecia can be arrested
by use of moderate potency topical steroids, but this is not a definite
finding. In cases where the condition is rapidly progressive, oral
prednisone or chloroquine may temporarily slow down the advancement
of the disease. Limited research on the use of moderate potency
topical steroids, intralesional steroids, topical retinoic acid
and oral isotretinoin and griseofulvin indicate that these drugs
have not been particularly effective, but these assumptions remain
unproven. Hormone replacement therapy does not alter the rate of
the progress of the disease.
Frontal fibrosing alopecia or postmenopausal frontal fibrosing
alopecia is a subset of cicatricial alopecia characterized by a
band of frontal or fronto-parietal hair recession and an overt thinning
or a complete loss of the eyebrows, characteristically observed
in women who are postmenopausal. The affected part of the scalp
is of variable width, and a shiny band of partial hair loss is obvious
above the forehead.
Frontal
fibrosing alopecia references
- Herrmann A, Bormann G, Marsch WC, Wohlrab J.
[Frontal fibrosing alopecia in two postmenopausal
women] J Dtsch Dermatol Ges. 2004 Aug;2(8):684-6. PMID:
16279232
- Moreno-Ramirez D, Camacho Martinez
F. Frontal fibrosing alopecia: a survey
in 16 patients. J Eur Acad Dermatol Venereol. 2005 Nov;19(6):700-5.
PMID: 16268874
- Jumez N, Bessis D, Guillot
B. [Frontal fibrosing alopecia is
not always post-menopausal] Ann Dermatol Venereol. 2005 Mar;132(3):263.
French. No abstract available. PMID: 15924052
- Kossard S, Shiell RC.
Frontal fibrosing alopecia developing after hair transplantation
for androgenetic alopecia. Int J Dermatol. 2005 Apr;44(4):321-3.
PMID: 15811087
- Tosti A, Piraccini BM, Iorizzo M, Misciali C. Frontal fibrosing
alopecia in postmenopausal women. J Am Acad Dermatol. 2005 Jan;52(1):55-60.
PMID: 15627081
- Vaisse V, Matard B, Assouly P, Jouannique
C, Reygagne P. [Postmenopausal frontal
fibrosing alopecia: 20 cases] Ann Dermatol Venereol. 2003 Jun-Jul;130(6-7):607-10.
French. PMID: 13679696
- Fiorucci
MC, Cozzani E, Parodi A, Rebora A.
Frontal fibrosing alopecia. Eur J Dermatol. 2003 Mar-Apr;13(2):203-4.
PMID: 12695141
- Dawn G, Holmes
SC, Moffat D, Munro CS. Post-menopausal
frontal fibrosing alopecia. Clin Exp Dermatol. 2003 Jan;28(1):43-5.
PMID: 12558629
- Naz
E, Vidaurrazaga C, Hernandez-Cano N, Herranz P, Mayor
M, Hervella M, Casado M. Postmenopausal
frontal
fibrosing alopecia. Clin Exp Dermatol. 2003 Jan;28(1):25-7.
PMID: 12558623
- Heyer M, Stemm AV,
Meigel W. [Band-like alopecia of the
forehead-hair zone. Postmenopausal frontal fibrosing alopecia]
Hautarzt.
2002 Nov;53(11):749-50. PMID:
12539783
- Claude V, Blanchet
P,
Grossin M, Henin D. [Postmenopausal
frontal fibrosing
alopecia. Report of 3 cases] Ann Pathol. 2002 Sep;22(4):328-30.
PMID: 12410158
- Stockmeier
M, Kunte C, Sander CA, Wolff H. [Kossard
frontal fibrosing alopecia in a man] Hautarzt. 2002
Jun;53(6):409-11. PMID: 12132298
- Faulkner CF, Wilson NJ, Jones SK. Frontal
fibrosing
alopecia associated with cutaneous lichen planus
in a premenopausal woman. Australas J Dermatol. 2002 Feb;43(1):65-7.
PMID: 11869213
- Camacho Martinez F, Garcia-Hernandez MJ, Mazuecos Blanca J.
Postmenopausal frontal fibrosing alopecia. Br
J
Dermatol.
1999 Jun;140(6):1181-2. PMID:
10354100
- Lee WS, Hwang SM, Ahn SK. Frontal
fibrosing alopecia in a postmenopausal woman. Cutis. 1997 Dec;60(6):299-300.
PMID: 9426971
- Kossard S, Lee
MS, Wilkinson B. Postmenopausal frontal
fibrosing alopecia: a frontal variant of lichen planopilaris.
J Am Acad Dermatol. 1997 Jan;36(1):59-66. PMID: 8996262
- Feldmann R, Harms M, Saurat JH. [Postmenopausal frontal fibrosing
alopecia] Hautarzt. 1996 Jul;47(7):533-6. PMID: 8926170
- Kossard S. Postmenopausal
frontal fibrosing alopecia. Scarring alopecia in a pattern
distribution. Arch Dermatol. 1994 Jun;130(6):770-4. Erratum
in: Arch Dermatol
1994 Nov;130(11):1407.
PMID: 8002649
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