2. OTHER CICATRICIAL ALOPECIA
FATEMEH MOKHTARI
Associate Professor of dermatology

3. CICATRICIAL (SCARRING) ALOPECIAS
Follicular epithelium has been replaced by connective tissue
In most cases, permanent injury of the follicular stem cell region has occurred
A few hair diseases demonstrate a biphasic pattern:
androgenetic alopecia
alopecia areata
traction alopecia

4. CICATRICIAL (SCARRING) ALOPECIAS
In primary cicatricial alopecia, the target of inflammation appears to be the follicle
In secondary cicatricial alopecia, the follicle is merely an “innocent bystander” in the disease process
Examples of secondary cicatricial alopecia include:
trauma (burns, radiation)
infiltrative processes (morphoea, scleroderma, sarcoidosis, neoplasias)
infections (bacterial, fungal, viral, mycobacterial)

5. Classification of Primary Cicatricial Alopecia
Is both confusing and controversial
Most of these diseases demonstrate some overlap in clinical and histologic features
In 2001, a North American Hair Research Society workshop developed a provisional classification to facilitate future discussion and research:
Lymphocytic
Neutrophilic
mixed lymphocytic and neutrophilic
Even advocates of this classification scheme, which is based on histologic findings, have found that it can fail to achieve reliable clinicopathologic correlation

6. Classification of Primary Cicatricial Alopecia
For the sake of simplicity, primary, inflammatory cicatricial alopecia can be divided into six diagnostic groups:
central centrifugal cicatricial alopecia
lichen planopilaris
discoid lesions of lupus erythematosus
acne keloidalis
dissecting cellulitis
cicatricial alopecia, not otherwise classified

7. Classification of Primary Cicatricial Alopecia
Older terms such as pseudopelade, pseudopelade of Brocq, and folliculitis decalvans are poorly defined
They are used in various ways by different authors and clinicians
Almost all of these older terms can be incorporated into one of the six categories already listed

8. CICATRICIAL (SCARRING) ALOPECIAS
Performing a scalp biopsy may be helpful:
establishing a diagnosis
assessing the degree of inflammation and injury to the stem cell region
Specimens should be at least 4 mm in diameter and extend into the fat
Ideally, two should be obtained – one for vertical sectioning, the other for horizontal sectioning
In the HoVert technique:
a 4 mm punch biopsy is transected ~1 mm below the skin surface in order to create an epidermal disc (for vertical sections) and a lower portion (for horizontal sections)

9. Central Centrifugal Cicatricial Alopecia

10. Central Centrifugal Cicatricial Alopecia
Clinical features
Slowly progressive, symmetric cicatricial alopecia centered on the crown or vertex
Symptoms may be:
mild
absent
most patients note only mild, episodic pruritus or tenderness of involved areas

11. Central Centrifugal Cicatricial Alopecia
Clinical features
Longstanding or severe disease can result in hair loss covering the entire crown of the scalp.
Pustules and crusting may be found in the minority of patients who:
suffer from rapidly progressive disease
the immune response of the patient to degenerating follicular components
folliculitis decalvans
a manifestation of bacterial superinfection
2- to 3-week course of antibiotics or systemic corticosteroids can temporarily eliminate the purulent component of CCCA.

12. Central Centrifugal Cicatricial Alopecia
Differential diagnosis
male and female pattern alopecia
lichen planopilaris
unusual cases of discoid lupus erythematosus
tinea capitis (kerion)
true bacterial infections of the scalp

13. Central Centrifugal Cicatricial Alopecia
Treatment
For relatively non-inflammatory disease:
longacting oral tetracycline (e.g. doxycycline, minocycline) plus a potent topical corticosteroid (e.g. clobetasol, fluocinonide)
Prolonged treatment for years is usually required for this chronic disease.

14. Central Centrifugal Cicatricial Alopecia
Treatment
For highly inflammatory cases (“folliculitis decalvans”):
an initial 10-week regimen of oral rifampin and clindamycin (both at 300 mg twice daily)
maintenance therapy (such as oral doxycycline plus topical clobetasol)
In small case series and case reports:
Nd:YAG laser
photodynamic therapy
TNF-α inhibitors

15. Discoid Lupus Erythematosus

16. Discoid Lupus Erythematosus
Clinical features
The diagnosis of DLE requires histologic confirmation and cannot be based solely on the clinical appearance of scalp lesions.
They may resemble classic discoid lesions elsewhere, with:
erythema
epidermal atrophy
dilated, plugged follicular ostia
alopecia
Central hypopigmentation and peripheral hyperpigmentation are commonly seen in dark-skinned individuals.
Although pruritus or tenderness is common, the condition may be asymptomatic

17. Discoid Lupus Erythematosus
Differential diagnosis:
alopecia areata
lichen planopilaris
linear morphea
central centrifugal cicatricial alopecia
Brocq’s alopecia

18. Discoid Lupus Erythematosus
Treatment
Oral antimalarial drugs (e.g. hydroxychloroquine) and corticosteroids (topical, intralesional, and oral)
If initiated early, a surprising amount of regrowth can occur.

19. Discoid Lupus Erythematosus
Antimalarials
Initial screening:
Ocular: Slit lamp and fundoscopic examination: assessment of visual acuity and visual field testing
Laboratory:
CBC
CMP
G6PD (selected cases)

20. Discoid Lupus Erythematosus
Antimalarials
Follow-up monitoring
Ocular:
repeat testing every 6 months for 1 year and then yearly
recent AAO guidelines:
baseline examination within the first year of use
annual screening after 5 years of continuous therapy, unless high-risk patient or symptoms arise
Laboratory:
CBC monthly for 3 months, then every 4–6 months
CMP after 1 and 3 months, then every 4–6 months

21. Discoid Lupus Erythematosus
Treatment
Other oral medications including:
mycophenolate mofetil
retinoids
thalidomide
azathioprine
low-dose weekly methotrexate
cyclosporine
TNF-α inhibitors
In a controlled study:
topical R-salbutamol
pulsed dye laser

22. A randomized controlled trial of R-salbutamol for topical treatment of discoid lupus erythematosus
The active substance is a sulfate salt of R-salbutamol in 0.5% concentration in an oil-in-water emulsion
 R‐salbutamol cream 0·5% (ASF‐1096; Astion, Copenhagen, Denmark)
Twice daily for 8 weeks
Efficacy was evaluated through scores of erythema, scaling/hypertrophy and induration as well as pain and itching
Application of R-salbutamol cream 0.5% was safe and well tolerated.
Br J Dermatol. 2009

23. Pulsed-dye laser as an adjuvant treatment for discoid lupus erythematosus: a randomized, controlled trial
Treatments with the PDL (595 nm) were delivered every four weeks for four consecutive months.
The patients were evaluated at weeks 0, 4, 8, 12, 16 and 24. Erythema index (EI) and Texture index (TI) were obtained 
Improvements of DLE can be achieved with PDL
J Dermatolog Treat. 2018

24. Acne Keloidalis

25. Acne Keloidalis Key features:
most common in black men, but can occur in women and Caucasians
a primary form of cicatricial alopecia
often occurs in conjunction with central centrifugal cicatricial alopecia

26. Acne Keloidalis Clinical features
Begins as small, smooth, firm papules admixed with occasional pustules on the occipital scalp and posterior neck
In a minority of patients, lesions are more numerous on the vertex and crown
With time, the papules resolve and leave small zones of alopecia
In many patients, the papules coalesce and form firm, hairless, keloid-like protuberant plaques that can be painful and cosmetically disfiguring
Abscesses and sinuses exuding pus may be present in advanced cases.

27. Acne Keloidalis Treatment
Treatment is easiest and most effective when instituted during the early (papular) phase.
Chronic use of a potent topical corticosteroid such as clobetasol and a long-acting oral antibiotic such as doxycycline
Laser-based hair removal
Large keloidal lesions requires surgical excision below the level of the hair bulbs

28. Dissecting Cellulitis of the Scalp

29. Dissecting Cellulitis of the Scalp
Introduction
It is part of the “follicular occlusion tetrad” that includes:
hidradenitis suppurativa
acne conglobata
pilonidal sinus
Isolated scalp disease is often seen.
Most commonly affects young adult men, especially black men, but can be seen (albeit rarely) in Caucasians and women.
Although follicular hyperkeratosis rather than infection is thought to play a primary role in pathogenesis, bacterial superinfection can occur.

30. Dissecting Cellulitis of the Scalp
Clinical features
Lesions begin as multiple, firm scalp nodules, most commonly on the mid and posterior vertex and upper occiput.
The nodules rapidly develop into interconnecting, boggy, fluctuant, oval and linear ridges that eventually discharge purulent material
There can be surprisingly little pain, and patients often seek help because of hair loss and a foul-smelling discharge.
Differential diagnosis
Tinea capitis
Folliculitis decalvans

31. Dissecting Cellulitis of the Scalp
Treatment
Isotretinoin (0.5–1.5 mg/kg daily until 4 months after achieving a clinical remission) may be an effective therapy although relapses are common.
Unfortunately, isotretinoin is not always effective.
TNF-α inhibitors have been used with success, but to date have not been critically studied.
Other treatment options include:
intralesional corticosteroids
oral antibiotics
oral high-dose zinc sulfate (135–220 mg TID)
Laser based hair removal
photodynamic therapy
radiation therapy
surgical approaches ranging from incision and drainage to excision with grafting

32. Cicatricial Alopecia, Not Otherwise Classified
“Burnt-out” or “end-stage” cicatricial alopecia
Brocq’s alopecia (pseudopelade of Brocq)
Folliculitis decalvans”
“Tufted folliculitis”
Traction Alopecia (End-Stage)

33. Burnt-out” or “end-stage” cicatricial alopecia
In patients with progressive hair loss, the diagnosis of “burnt-out” or “end-stage” cicatricial alopecia invites additional biopsy specimens
Although the patient’s disease may truly be in remission, often the histologic pattern of “end-stage” disease is due to sampling of an older, inactive portion of the lesion.

34. Brocq’s alopecia (pseudopelade of Brocq)
Is a source of much confusion and should be abandoned.
Brocq’s alopecia is not a distinct disease but rather a clinical pattern of end- stage cicatricial alopecia
This pattern can be seen in:
lichen planopilaris
DLE
other forms of cicatricial alopecia
If a definitive diagnosis of another form of cicatricial alopecia can be made, the term “Brocq’s alopecia” cannot be used.

35. Brocq’s alopecia (pseudopelade of Brocq)
Is uncommon and usually affects Caucasian adults
Results in irregularly shaped and often widely distributed and grouped bald patches on the scalp
Patients with exclusive crown or vertex involvement may actually represent examples of “burnt-out” central centrifugal cicatricial alopecia (CCCA).
In most cases of Brocq’s alopecia, the expected histologic findings are those of a “burnt-out” cicatricial alopecia.

36. Folliculitis decalvans
Is used in different ways by different authors, and so must be redefined each time the term is used.
Usually, it is applied to highly inflammatory forms of cicatricial alopecia, where inflammatory, follicular papules and pustules dominate the clinical picture.
Often (but not always) Staphylococcus aureus can be grown from pustular or crusted lesions.
Some authors believe that a primary staphylococcal infection of the scalp is the cause of folliculitis decalvans

37. Folliculitis decalvans
Mild cases can be managed with:
topical clindamycin
antiseptic shampoos
Treatment of severe disease is more complicated and may involve maintenance therapy with oral tetracyclines
In one series of patients, long-term remissions were observed with a combination of oral rifampin and clindamycin

38. Tufted folliculitis
The “tufting” seen in this pattern of hair disease is common to several forms of scarring alopecia.
It is not a specific disease, but an end stage of several different conditions
Another name for this phenomenon is polytrichia.

39. Tufted folliculitis Tufting is fairly common in cases of CCCA
It can occasionally be seen in a wide variety of other disorders, including:
acne keloidalis
dissecting cellulitis
pemphigus
thermal burns
inflammatory tinea capitis

40. Traction Alopecia (End-Stage)
Is a biphasic form of hair loss.
Initially, the hair loss is temporary, hair regrowth can occur, and the condition behaves like a non-cicatricial form of alopecia.
However, if excessive traction is maintained for years, the hair loss may eventually become permanent (end-stage or “burnt-out”).
There may be a lag period of a decade or more between the period of traction and the onset of permanent hair loss.

41. Summary CICATRICIAL (SCARRING) ALOPECIAS Primary Secondary
Classification of Primary Cicatricial Alopecia
central centrifugal cicatricial alopecia
lichen planopilaris
discoid lesions of lupus erythematosus
acne keloidalis
dissecting cellulitis
cicatricial alopecia, not otherwise classified
“Burnt-out” or “end-stage” cicatricial alopecia
Brocq’s alopecia (pseudopelade of Brocq)
Folliculitis decalvans”
“Tufted folliculitis”
Traction Alopecia (End-Stage)

42. Summary
Biopsy
Clinical features
Differential diagnosis
Treatment