Download Cicatricial (scarring) alopecia: an overview and a clinical approach

Clinical review
Cicatricial (scarring) alopecia:
an overview and a clinical approach
to diagnosis
Yi Zhen Chiang, Firas Al-Niaimi
Scarring alopecia, also known as cicatricial alopecia, forms an important group of disorders whereby there
is irreversible damage to hair follicles resulting in scarring and permanent hair loss. The hair follicles can be
destroyed directly by a primary process (primary cicatricial alopecia) or secondary to an underlying disease
or an external agent (secondary cicatricial alopecia). A diagnosis of scarring alopecia can be achieved on
both clinical and histological grounds. Patients with cicatricial alopecia often have significant psychosocial
impact and dermatology nurses can play an important role in the care for these patients.
Introduction
Scalp hair is an important component of
identity, body image and self-esteem, and
is often used to express personality and
sexuality. It is therefore not surprising that
alopecia (hair loss) can lead to significant
psychosocial distress, psychiatric disorders,
marital problems and career-related
problems (Hunt, 2005). Alopecia can
be cicatricial (scarring) or non-cicatricial
(non-scarring).This review will focus on
cicatricial alopecia with an aim to providing
a systematic approach to the assessment
of patients with suspected cicatricial
alopecia and an overview of the different
types of cicatricial alopecia.
Cicatricial alopecia forms a rare,
but important group of disorders
characterised by permanent destruction
of hair follicles, resulting in scarring and
permanent hair loss. The hair follicle
can be destroyed by a primary process
aimed directly at the hair follicle (primary
cicatricial alopecia (PCA)), or secondary
to a generalised destructive process
within the skin, which ultimately destroys
the hair follicle’s capacity for regeneration
(secondary cicatricial alopecia) (Harries,
2009).
pathogenesis of primary cicatricial
alopecias is not known, but is likely to
result from an irreversible damage to the
epithelial hair follicle stem cells that reside
in the hair follicle ‘bulge’. This is suggested
by the pattern of inflammation seen in
cicatricial alopecia, which is around the
‘bulge’, as opposed to non-cicatricial
alopecia, which is around the hair bulb
(Harries, 2009).
Secondary cicatricial alopecia develops
as a result of an underlying process or
an external agent. Potential causes are
inflammatory/autoimmune diseases (eg,
scleroderma, sarcoidosis), infections (eg,
tinea capitis), neoplastic processes (eg,
primary skin cancer, metastatic carcinoma),
and physical agents (eg, ionising radiation,
thermal burn) (Headington, 1996).
Classification
The North American Hair Research
Society (NAHRS) has produced
a working classification of primary
cicatricial alopecia. The classification is
based primarily on the predominant
inflammatory infiltrate found on scalp
biopsy, as shown in Table 1 (Olsen, 2003).
Clinical assessment
Patients with cicatricial alopecia often
present fairly late due to the subtle,
relentless progression of the disease.
Diagnosis can be challenging due to the
evolving clinical and histological features
over time, and the overlapping clinical
and histological features seen in one
condition with the other (Harries, 2009).
Nevertheless, a systematic approach to
establish an accurate diagnosis is a vital first
step in successful management.
History
Patients with cicatricial alopecia may
present with an acute or gradual onset
of hair loss and symptoms. Common
symptoms include pain, irritation, itching
and discharge. A full history should include
the patient’s ethnic origin and age as some
Table 1.
The North American Hair Research Society classification of primary cicatricial
alopecias (Olsen EA, 2001).
The epidemiology of PCA has been
reported to range from 3.2% to 7.3%
(Whiting, 2001, Tan, 2004). The exact
Inflammatory infiltrate
Diagnosis
Lymphocytic
Lichen plano pilaris and variants; chronic cutaneous lupus erythematosus;
pseudopelade of Brocq; central centrifugal cicatricial alopecia
Yi Zhen Chiang is a Specialty Trainee in
Dermatology at Birmingham City Hospital.
Firas Al-Niaimi is a Fellow in Mohs Surgery
and Laser, St John’s Institute of Dermatology,
London
Neutrophilic
Folliculitis decalvans/Tufted folliculitis; dissecting cellulitis of the scalp
Mixed
Non-specific
Folliculitis acne keloidalis
End stage of scarring
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Figure 1. Lichen planopilaris (LPP).
Figure 2. Pseudopelade of Brocq
(PB).
conditions appear more commonly in
certain racial groups and age (Han, 2006).
A complete and focused history should
aim to elicit the following points:
8 Ethnic origin
8 Age
8 Onset
8 Progression
8 Local and systemic symptoms
8 Other medical problems (eg, presence
of autoimmune diseases or associated
inflammatory skin conditions such as
lupus erythematosus (LE), lichen planus
(LP), infections, malignancies)
8 Previous treatment (including any
previous exposure to thermal burns,
radiation treatment)
8 Previous hair care practice (eg, use of
hot combs, excess traction)
Examination
A crucial first step in clinical examination is
to confirm the presence of scarring on the
areas of hair loss, which is characterised
by the loss of follicular ostia (openings).
Additional clues to scarring are epidermal
atrophy, irregularly spaced hair shafts and
hair tufting (multiple hairs emerging from
a single follicular ostium) (Han, 2006).
A good light, aided by a magnifying lens
or dermatoscope, is essential in the
examination of the scalp.
Scalp inflammation often indicates
active disease, and the signs of an active
disease include erythema, scaling, crusting,
pustules, scalp bogginess, and a positive pull
test with anagen bulbs seen on the hair
mount (Harries, 2009, Han, 2006).
The following features should be
recorded:
8 Pattern of hair loss and extent of scalp
involvement
8 Presence or absence of scarring
8 Associated skin changes on the scalp
(eg, erythema, scaling, atrophy, change
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Clinical review
Figure 3. Dissecting cellulitis of the
scalp (DCS).
in pigmentation, scalp bogginess,
pustules, crusting)
8 Other area(s) of hair loss
8 Signs of skin disease elsewhere (eg,
lichen planus, lupus erythematosus).
Investigations
Samples for microscopy, culture and
sensitivity (bacterial and fungal) are useful
if there are signs of infection such as
crusting, scaling, pustules or scalp bogginess.
Pustules, however, are not always
associated with infections, and can be seen
in normal scalps (Harries, 2009).
A scalp biopsy is often essential in
the assessment of cicatricial alopecia. It
will help to confirm scarring (if there
is any clinical doubt), and to identify
the secondary cause of cicatricial
alopecia, or to confirm the diagnosis of
a primary cicatricial alopecia based on
the predominant inflammatory infiltrate
involved (as shown in Table 1).
Diagnosis
The major forms of primary cicatricial
alopecia are lichen planopilaris (LPP)
(Figure 1) and variants — frontal fibrosing
alopecia (FFA) and Graham-Little
syndrome (GLS); chronic cutaneous lupus
erythematosus (CCLE); pseudopelade of
Brocq (PB) (Figure 2); central centrifugal
cicatricial alopecia (CCCA); folliculitis
decalvans (FD); dissecting cellulitis of the
scalp (DCS) (Figure 3); tufted folliculitis
(TF); and acne keloidalis nuchae (AKN)
(Figure 4) (Ross, 2005).
Lichen planopilaris and variants
LPP is characterised by multiple patches of
permanent alopecia distributed over the
central scalp, mostly affecting middle-aged
women. Mild to moderate itching has been
reported. Scaling and erythema around
the follicles in the expanding areas of
alopecia are commonly seen (Ross, 2005).
Figure 4. Acne keloidalis nuchae
(AKN).
FFP is a variant of LPP and presents
typically with a band-like symmetrical
recession of the frontal hairline.The
condition is common in post-menopausal
women. GLS is another variant of LPP
and is characterised by the triad of patchy,
progressive scarring alopecia, non-scarring
alopecia of axillary and pubic hair, and
widespread horny follicular papules
(keratosis pilaris) on trunk and limbs
(Ghislain, 2006).
Chronic cutaneous lupus erythematosus
A third of the cases of CCLE have scarring
alopecia.This condition is more common
in adult women. It commonly affects
the central scalp with associated skin
changes such as erythema, scaling, follicular
plugging, change in pigmentation, and skin
atrophy (Annessi, 1999, Harries 2009). In
contrast, diffuse and non-scarring hair loss
is frequently observed in systemic lupus
erythematosus (Cardinali, 2000).
Pseudopelade of Brocq
It is still not clear whether PB is a disease
in its own right or just the end stage of a
different scarring process. However, it has a
characteristic clinical picture.Typically, there
are multiple, variably sized, white to skincoloured plaques on the vertex (known
as ‘footprints in the snow’), and they are
largely asymptomatic (Braun-Falco, 1986,
Dawber, 1992).
Central centrifugal cicatricial alopecia
CCCA is often seen in women of African
descent and has been referred to as
‘follicular degeneration syndrome’ or
‘hot comb alopecia’ (Sperling, 1992).The
disorder commonly starts over the vertex
and spreads symmetrically and centrifugally.
The hair loss is often incomplete with
groups of hairs remaining in the area of
scarring. Symptoms are usually absent
and there is no clinical evidence of
inflammation (Harries, 2009).
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Clinical review
Folliculitis decalvans, tufted folliculitis,
dissecting cellulitis of the scalp and acne
keloidalis nuchae
These conditions form a group of scarring
alopecia that present with discharging
material.They affect men and women
from young adulthood to middle-age.The
vertex is most commonly affected.The
scarred areas are often hypertrophic with
features of pustules, crusting and tufts.TF is
often thought to be a variant of FD. DCS is
seen almost exclusively in males of African
descent.There are typically multiple painful
interconnecting scalp nodules, fluctuant
nodules which may discharge purulent
material, and the presence of sinus tracts
(Brooke, 2001). AKN commonly involves
the occipital scalp. Like DCS, AKN tends to
affect young men of African descent. Keloid
plaques are seen, with secondary infection
suggested by the presence of pustule
formation, pain and discharge (Sperling,
2000, Dinehart, 1989).
Management
Management of cicatricial alopecia is
challenging as hair regrowth in scarred
areas hardly ever occurs.The principle
aim of treatment is to reduce symptoms
and to stop or slow progression of the
scarring process. It is important to ensure
that patients understand the aims of their
treatment and are provided with a realistic
expectation.
As a general rule, immunosuppressants
such as potent topical corticosteroids,
intralesional triamcinolone acetate, oral
corticosteroids, and antimalarials (eg,
hydroxychloroquine) are useful for the
lymphocyte-predominant sub-group
of primary cicatricial alopecia; while
antimicrobials (eg, tetracyclines) and
dapsone are useful for the neutrophilpredominant sub-group. Patients with
completely stable or ‘burned-out’ cicatricial
alopecia may benefit from scalp reduction
surgery and, possibly, hair transplantation
(Harries, 2008). Damaging hair care
practice such as tight hair braiding, use
of chemical relaxers, glued-in or sewnin hair weaves should be stopped.The
underlying cause of a secondary cicatricial
alopecia should be identified and treated
accordingly.
As hair loss is associated with
significant psychosocial morbidity,
34
management of these patients should
also include addressing their psychosocial
aspects. Patients can be referred to a
psychologist for counselling and ways
to deal with the psychological impact of
hair loss if needed. Regular monitoring
of disease progression and adequate
provision of information can help relieve
unnecessary anxiety. Dermatology nurses
who care for these patients can play a
role in providing continuous support,
information on scalp and hair care
practice, techniques of scalp coverage
and camouflage, and to ensure that these
patients have a good understanding
about the nature and treatment of their
condition. Useful information on cicatricial
alopecia can be obtained from the British
Association of Dermatologists (BAD)
website and various patient support
groups (Table 2) (Hunt, 2005).
Conclusion
Cicatricial alopecia forms an important
group of disorders that result in scarring
and permanent hair loss. An early diagnosis
can be achieved by a systematic approach
to the clinical assessment. Patients with
cicatricial alopecia often have significant
psychosocial impact and management of
these patients should address both their
physical and psychological aspects. DN
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Table 2.
Useful resources for patients and
healthcare professionals.
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