LICHEN PLANOPILARIS CHRONIC CUTANEOUS LUPUS ERYTHEMATOUS CENTRAL CENTRIFUGAL CICATRICAL ALOPECIA PSEUDOPELADE OF BROCQ ALOPECIA MUCINOSIS KERATOSIS PILARIS SPINULOSA DECALVANS

1. TUTORIAL PRESENTATION
CICATRICIAL ALOPECIA

2. Definition:Permanent area of hair loss associated with
destruction of hair follicles
 Pilosebaceous structures are replaced by fibrous tracts
 Classified as:
1. Primary
2. Secondary

Trauma

Sclerosing disorders

Granulomatous disorders

Infections

Neoplastic
3. Developmental/Hereditary

PCA more common than SCA (4:1)


3. ANOTHER CLASSIFICATION PROPOSED TO FACILITATE
DETERMINATION OF MOST SUITABLE SURGICAL CORRECTIVE
THERAPIES FOR

CA :
stable 


Traumatic
Aplasia cutis
CCCA
unstable –






Lymphocytic
Neutrophilic
Mixed
Infections
Congenital
Neoplastic

4. PATHOGENESIS






HFSCs destruction theories
Impairment of self maintenance of HFSCs
Alteration of lipid metabolism
Neurogenic inflammation theory
Environment factors
Genetic factors

5. PPAR DEFICIENCY CAUSES LOSS OF PEROXISOME BIOGENESIS, DEREGULATES LIPID
METABOLISM, AND PRODUCES PROINFLAMMATORY LIPIDS THAT TRIGGER INFLAMMATORY
RESPONSE THAT IN TURN CAUSES TISSUE DAMAGE AND PERMANENT HAIR LOSS

6. 
NORMAL
IN CICATRICIAL ALOPECIA

7. APPROACH TO THE PATIENT

History





Clinical Findings



Onset
Presence of- pruritus, irritation, pain, erythema and/or drainage
from the scalp
Evaluate for autoimmune disease, systemic illness, infections,
neoplasms, associated inflammatory skin disease and radiation
treatment or burns
Drug intake
loss of follicular ostia
erythema, scaling, pustules, scalp bogginess and compound
follicles (polytrichia)
Biopsy

8. Non-scarring hair
Incidence
Erythema/scaling/pustules
More common
Scarring hair loss
Less common
-/+
+
Atrophy
absent
present
Loss of follicular openings
absent
present
Tufted hair
absent
present
Course
regrowth is quite
common
Prognosis
generally favourable
no regrowth
generally unfavourable

9. PRIMARY CICATRICAL
ALOPECIA
Diagnosis in which lymphocytes
predominates includes:
LICHEN PLANOPILARIS
CHRONIC CUTANEOUS LUPUS ERYTHEMATOUS
CENTRAL CENTRIFUGAL CICATRICAL ALOPECIA
PSEUDOPELADE OF BROCQ
ALOPECIA MUCINOSIS
KERATOSIS PILARIS SPINULOSA DECALVANS

10. Classic lichen planus
40% of pt have skin manifestations
C/Fs: violaceous papules,erythema scaling
Papules replaced by follicular plugs
Plugs shed and finally atrophic,smooth,scarred
area remains
Pt commonly presents with pseudopelade like
patches
Frontal Fibrosing
Alopecia
Resembles AGA with frontal recession
C/Fs:perifollicular erythema and
hyperkeratoses at marginal hairline
Slow progressive disease
Typically occurs in Post-menopausal
women
GRAHAM-LITTLE PICCARDI LASSUEUR SYNDROME
AFFECTS WOMEN BETWEEN 30-70 YRS
SYNDROME CHARACTERIZED BY:PROGRESSIVE CICATRICIAL ALOPECIA OF
SCALP,NON SCARRING ALOPECIA OF AXILLA AND PUBIC AREA AND KERATOSIS
PILARIS

11. Central centrifugal
cicatricial alopecia
hot comb alopecia, follicular
degeneration syndrome, pseudopelade
in African Americans central elliptical
pseudopelade
Premature disintegration of inner root
sheath epithelium occurs
Begins as single focus over vertex of
scalp and then spread centrifugally
Pseudopelade
Idiopathic,chronic,slowly
progressive
Patchy cicatricial alopecia that
occur without any evidence of
inflammation
footprints in the snow

12. Chronic Cutaneous Lupus
Erythematosus
Keratosis follicularis
spinulosa decalvans
30% have skin manifestation
Erythema, scaling and pigmentary
changes are more pronounced
Follicular plugging and adherent
scale may be present.
The “carpet tack” sign may be
elicited with retraction of scale
revealing keratotic spikes that
correspond to follicular openings on
undersurface
X-Linked recessive
SSAT gene defect
Erythema, plugging of eyebrow
follicles follicular hyperkeratosis &
prominent cuticles
Ocular signs include blepharitis,
ectropion, corneal dystrophy and
photophobia
Focal PPK may be present

13. 
Diagnosis in which neutrophils predominates
includes:
FOLLICULITIS DECALVANS
DISSECTING CELLULITIS OF SCALP

14. Folliculitis decalvans
-Recurrent crops of follicular
pustules that result in permanent
epilation
-Staph aureus may be grown from
pustules
-Pustular folliculitis followed by
rounded patches of alopecia
develop surrounded by crusting and
few follicular pustules.
-Successive crops of pustules
appear and are followed by
progressive destruction of affected
follicles
-Tufted folliculitis variant of
folliculitis decalvans where
circumscribed areas of scalp
inflammation heal with scarring
characterized by tufts of up to 15
hairs emerging from single orifice

15. Dissecting cellulitis of the scalp
Perifolliculitis capitis abscedens et suffodiens






Perifolliculitis of scalp, deep and
superficial abscesses in dermis, sinus
tract formation and extensive scarring
Aetiology:staphylococci, streptococci and
Pseudomonas may be cultured from
various lesions
C/Fs:Painful, firm, skin-coloured nodules
develop near vertex
Confluent nodules form tubular ridges with
an irregular cerebriform pattern
Progressive scarring and permanent
alopecia occur
Chronic condition with frequent acute
exacerbations.

16. 
Diagnoses in which a mix of cell types predominate
are as follows:
ACNE KELOIDALIS
ACNE NECROTICA
EROSIVE PUSTULAR DERMATOSES OF SCALP

17. Acne keloidalis
It occurs in males after puberty
between the ages of 14 -25 yrs
C/Fs:Pts present with pustules,
alopecia and hypertrophic scarring
on posterior neck
Friction from the collar is often
incriminated
Process begins with penetration
of cut hair into
the skin as in pseudofolliculitis
Acne necrotica
More frequent in men than in women
30- 50 yrs
C/Fs:red itchy acneiform papules arise
spontaneously on the front and sides of
scalp
papules are usually centered around
pilosebaceous unit
Often umbilicated and rapidly transformed
by necrosis into an adherent haemorrhagic
crust which separates after 3 or 4 weeks to
leave a permanent varioliform scar

18. Erosive pustular dermatosis of the scalp






Particularly affects the elderly
Precipitating factors:local trauma
sundamage,surgery, cryosurgery
skin grafting and radiation
therapy
Initially, a small area of scalp
becomes red, crusted and
irritable
crusting and superficial
pustulation overlie a moist,
eroded surface
As condition extends areas of
activity coexist with areas of
scarring.
Squamous carcinoma has
developed in scars

19. SECONDARY CICATRICIAL
ALOPECIA

20. GRANULOMATOUS DISORDERS
Necrobiosis lipoidica, granuloma
annulare and sarcoidosis





C/F:The oval atrophic plaques
on the shins but may be seen
on other parts of body including
scalp.
The patches are glazed,
yellowish often with
conspicuous telangiectasia
Scarring may be dense.
Clinical features in scalp vary
from large plaques of cicatricial
alopecia to multiple small areas
of scarring
Cutaneous sarcoidosis may
produce plaques or nodules on
scalp

21. SCLEROSING DISORDERS
Circumscribed scleroderma
and linear morphoea
rare in the scalp
‟en coup de sabre‟ morphoea – is
more common
Cicatricial pemphigoid
Women > men
disease predominantly affects ocular
and/or genital mucous membrane
skin is involved in 40–50%
scalp involved in 10% of cases

22. TRAUMATIC
Traction alopecia

Common in Afro-Caribbean hair
styles

Due to sustained pull on hair
roots

Folliculitis , hair casts
reduction in hair density with
vellus hairs and sometimes
broken hairs

Hair loss begins in temporal
regions and in front of and
above the ears but may involve
other parts of scalp
Medical trauma

CHILD: Scalp electrodes or
infusion or forceps delivery
or uterine rings in neonate
can result in trauma.

ADULT:Brain surgery,
gynaecological surgery in
Trendelenburg position

23. TRICHOTILLOMANIA









Behavioural disorder characterized by compulsive hair pulling
Trichoteiromania : Compulsive hair rubbing
Trichotemnomania : Compulsive hair cutting
Hair is plucked most frequently from one frontoparietal region
Patch of hair loss
bizarre or angular pattern in which hairs are
twisted and broken at various distances from clinically normal scalp
H/P: Numerous empty canals , clefts in hair matrix, intraepithelial and
perifollicular haemorrhages and intrafollicular pigment casts
Some follicles are severely damaged
Follicular epithelium is separated from connective tissue sheath
Trichomalacia - Injured follicles may form only soft, twisted hair

24. Syphilitic alopecia.
The scalp has moth-eaten appearance, eyebrow hair
is absent there is rash on the cheek
Trichotillomania. Hairs are thin
and of different lengths
TRACTION ALOPECIA. TRACTION FOLLICULITIS
IS COMMONLY ASSOCIATED

25. TRACTION ALOPECIA
DERMATOPHYTE:INFECTION

26. DIAGNOSIS

Dermoscopy/Trichoscopy - first-line, noninvasive method

Absence of follicular ostia in 100% cases even if it is not evident
clinically
FFA :loss of orifices, perifollicular scale and feeble perifollicular
erythema
Folliculitis decalvans :existence of micropustules and/or hair tufting
with >=6 hairs
DLE: follicular red dots
LPP: hair tufting, violaceous-blue interfollicular area, corresponding
to pigment incontinence
Lipedematous alopecia: linear area of telangiectasia within scalp
creases, possibly caused by compression of the superficial blood
capillaries
Scalp sarcoidosis: orange spots seen(round, well-formed
granulomas in superficial dermis)
Traction alopecia: Hair casts








29. Reflectance confocal microscopy
Microscopic imaging of superficial layers of skin down to
superficial reticular dermis with resolution at cellular level
close to conventional histopathology
 May also help in choosing most appropriate biopsy site
for more informative histology

Histopathology
Direct immunofluorescence
Microarray analysis

30. LUPUS ERYTHEMATOUS: H/P:VACUOLAR INTERFACE ALTERATION OF
FOLLICULAR EPITHELIUM, SCATTERING OF DYSKERATOTIC KERATINOCYTES, VARIABLY
DENSE PERIADNEXAL, PERIFOLLICULAR (UPPER PORTION), PERIVASCULAR
AND INTERSTITIAL LYMPHOCYTIC INFILTRATE WITH DERMAL MUCIN, ATROPHY OF
SEBACEOUS GLANDS AND FOLLICULAR PLUGGING. EPIDERMIS MAY BE ATROPHIED
WITH VACUOLAR INTERFACE CHANGES. CONCENTRIC LAMELLAR FIBROSIS AROUND
THE FOLLICLE IN END STAGES
follicular plugging, superficial,deep perivascular and
periappendageal lymphocytic infiltrate
There is linear staining of deposits of complement
(C3), IgM and IgG on the basement membrane in
more than 80% of cases of LE

31. LICHEN PLANUS: ACTIVE : FOLLICULAR LYMPHOCYTIC INTERFACE
DERMATITIS WITH DENSE BAND LIKE LYMPHOCYTES AROUND UPPER FOLLICLE
&
INFUNDIBULUM OBSCURING DEJ, INFUNDIBULAR HYPERKERATOSIS AND
HYPERGRANULOSIS, CYTOID BODIES SCATTERED ALONG THE BMZ, ABSENT OR
ATROPHIC SEBACEOUS GLANDS WITH OR WITHOUT PIGMENTARY INCONTINENCE.
END –STAGE : LONGITUDINAL TRACTS OF FIBROSIS,LAMELLAR FIBROSIS,EPIDERMAL
ATROPHY
DIF:„SHAGGY‟ OR „PATCHY‟ DEPOSITION OF FIBRINOGEN AND CLUMPED IGM OR LESS
COMMONLY IGA AND C3 DEPOSITS ARE SEEN ALONG FOLLICULAR BMZ

32. Pseudopelade of Brocq


Variably dense perifollicular
lymphocytic infiltrate in early
stage, followed by eccentric
atrophy of follicle
infundibular epithelium,
concentric lamellar fibrosis
around upper follicle and
loss of sebaceous gland in
later stage
Elastin stains reveal dense
elastic tissue cuffing a broad
fibrotic follicular tract in
advanced disease
thinned out epidermis with total
loss of hair follicles, replaced by
collagen

33. 
Central centrifugal cicatricial alopecia :Earliest feature is
premature disintegration of IRS resulting in outward migration of hair
shaft through ORS at level of isthmus. Lamellar fibroplasias and
lymphocytic inflammation surround the follicle at this level, resulting in
follicular destruction and fibrous tract formation.

Alopecia mucinosa :Mucinous degeneration of ORS and
sebaceous glands. A perifollicular lymphocytic infiltrate often with
eosinophils and histocytes

Keratosis follicularis spinulosa decalvans :Compact
hyperkeratosis, hypergranulosis of upper follicular epithelium with
superficial intrafollicular and peri-follicular edema in early stage whereas
in advanced stage there is concentric perifollicular, horizontal adventitial
lamellar fibrosis and scarred follicular tracts

34. 



Folliculitis decalvans: Acneiform dilatation with perifollicular
neutrophilic inflammation- later mixed inflammatory infiltrate of
neutrophils, lymphocytes, plasma cells. Follicular rupture,foreignbody giant cell granuloma formation around exposed hair shaft
fragments. In burnt outstage, follicular and adventitial fibrosis is seen
Dissecting cellulitis of scalp: Infundibular acneiform distention with
intrafollicular and perifollicular neutrophilic infiltration, abscess
formation,sinus tracts
Acne keloidalis : perifollicular and intrafollicular lymphoplasmacytic
infiltrate at the level of sebaceous glands -complete follicular
destruction occurs with loss of sebaceous glands and dermal fibrosis
Acne necrotica:dense perivascular and perifollicular lymphocytic
infiltrate with prominent sub-epidermal edema. Necrosis of individual
keratinocyte is seen initially and is followed by confluent necrosis of
the central follicle and interfollicular epidermis

35. TREATMENT

Aim of treatment currently focuses reduction of
symptoms and to reduce or stop progression of
disease.
LMPCA with immunosuppression
 NMPCA with antimicrobials or dapsone


36. DLE
First line
 Class I or II potent topical
corticosteroids
 I/L steroid inj (10 mg/ml max 2 ml,
every 4-6 weeks)
 Results are assessed till 8 weeks, if no
response shift to next level
Second line
 Antimalarials (HCQ 200-400 mg/day,
clinical effect in 4-8 weeks, continued
till 3-6 mths)
 Oral corticosteroids (1 mg/kg, for initial
actively progressing disease, tapered
over 8 weeks)
 Retinoids (acitretin and Isotretinoin 1040 mg/day)
Third line
 Thalidomide, topical
immunomodulators , oral vit E, gold,
dapsone,MMF,methotrexate,
azathioprine,clofazamine,systemic or
intralesional INFα2, monoclonal antiCD4 antibodies, topical 5-FU, topical
tazarotene imiquimod.
LPP
First line

Potent topical corticosteriods

I/L Triamcinolone acetonide
Second line

Oral corticosteroids

Oral cyclosporine(4-5 mg/kg for 4-6
mths)

Topical cyclosporine (oily solution,
applied twice daily for initial 3 mths and
once daily for further 3 mths)

Oral tetracycline
Third line

Retinoids ,Antimalarials,MMF(500 mg
twice daily)

Others: Thalidomide,griseofulvin,low
molecular weight heparin (s/c injections
3 mg once weekly), exicmer laser
Newer therapies

PPARγ agonist like thiozolidinediones

37. FFA






Intralesional triamcinolone acetonide
Finasteride (2.5 mg OD)
Oral corticosteroids
Antimalarials
Topical corticosteroids with topical
minoxidil
Oral retinoids
GLP




Topical and intralesional
corticosteroids
Oral ciclosporin
Systemic corticosteroids
Topical tacrolimus
PPB

Potent topical corticosteroids(±)
Alopecia mucinosa
First line
 Potent topical corticosteriods
Second line
 I/L triamcinolone
 Minocycline
 Dapsone
Third line
 Systemic steroids,isotretinoin,
antimalarials,PUVA,interferon α2b+Interferon γ,superficial X-rays
KFSD




Oral antibiotics
Dapsone
Oral retinoids
Laser epilation

38. FD
First line
 Oral±topical antibiotics
Second line
 Oral rifampicin (300 mg BD)+oral
clindamycin (300 mg BD)
 Rifampicin+ (doxycycline/ciprofl
oxacin/clarithromycin)
 Oral rifampicin+topical antibiotics
Third line
 Oral fusidic acid
 Oral zinc
 Dapsone
 Oral cyclosporine
 Excision,laser,radiotherapy,i/m
Human immunoglobulin
Dissecting cellulitis of scalp
First line
 Oral isotretinoin
 Oral isotretinoin+i/l
triamcinolone acetonide
Second line
 Oral antibiotics+topical
antibiotics/topical retinoids
 Aspiration and i/l triamcinolone
acetonide
Third line
 Low dose corticosteroids
 Colchicine
 Dapsone
 Excision and skin grafting
 Lasers and radiotherapy

39. Acne necrotica varioliformis
Acne keloidalis nuchae
First line
 Potent topical steroid
 Oral antibiotics+topical
steroids/intralesional
triamcinolone
Second line
 Surgical excision
 CO2 laser
 Diode laser hair epilation
Third line
 Radiotherapy
 Isotretinoin
Oral antibiotics
 Oral isotretinoin
 I/L triamcinolone

Erosive pustular
dermatosis of scalp
 Topical corticosteroids
 Topical
immunomodulators
 Calcipotriol cream
 Oral Isotretinoin

40. 
Surgical treatment of scarring alopecia includes :

hair transplantation
 scalp reduction or alopecia reduction surgeries
 tissue expansion
 flap surgeries

41. CONCLUSION

CA is „trichology emergency‟ situation, in which lack
of prompt and early treatment will lead to the
inevitable loss of hair follicles along with permanent
scarring.

Newer pathogenesis has given the platform for
development of emerging treatment modalities.

But still great deal of research is required in this
field, may be developing viable stem cell therapies
or bioengineered human hair follicles are the
answers to it in future

42. THANK YOU