Keywords
cicatricial alopecia (CA) - hair transplant - hair follicle - frontal fibrosing alopecia
(FFA) - central centrifugal cicatricial alopecia (CCCA)
Introduction
Cicatricial alopecia (CA) or scarring alopecia is a group of uncommon inflammatory
hair loss disorders characterized by permanent destruction of hair follicles. Clinically,
there is a loss of visible follicular ostia over the scarring area, with or without
epidermal atrophy; histologically, fibrous tracts replace the pilosebaceous structures.[1]
[2]
[3]
[4]
[5] This leads to permanent hair loss in the scarred zone. It can involve any area on
the scalp, including the facial regions. The available pharmacological treatments
can prevent the progression of the disease to some extent. Hair transplant (HT) for
CA is often explored ([Figs. 1]
[2]
[3]) but many critical questions regarding the time of surgery, surgical technique,
goal to achieve, how to decide the disease remission, and whether the surgical process
might trigger the relapse of the disease remain unanswered. This review intends to
summarize the available literature on HT in CA and come out with recommendations for
the same.
Fig. 1 (a) Preprocedure picture, (b) postprocedure picture.
Fig. 2 (a) Preprocedure picture, (b) postprocedure picture.
Fig. 3 (a) Preprocedure picture, (b) postprocedure picture.
Classification
The causes of CA are broadly classified as primary, secondary and hereditary or developmental
defects.[4]
[5]
[6] In primary cicatricial alopecia (PCA), there is irreversible hair loss from the
affected site on the scalp, and the inflammatory cells target and destroy the stem
cells in the bulge area of hair follicles.[4] Based upon the histopathological picture, the CAs are divided mainly into lymphocyte-mediated
primary cicatricial alopecia (LMPCA), neutrophil-mediated primary cicatricial alopecia
(NMPCA), and mixed CA[7] ([Table 1]).
Table 1
Classification of Cicatricial alopecia
|
Primary CA
|
Secondary CA
|
|
Lymphocyte-associated:
CCLE
LPP
Classical type
Frontal fibrosing alopecia
Graham–Little syndrome
Classic pseudopelade (Brocq)
CCCA
Alopecia mucinosa
Keratosis follicularis spinulosa decalvans
Neutrophil-associated:
Folliculitis decalvans
Dissecting cellulitis/folliculitis (perifolliculitis abscendens et suffodiens)
Mixed inflammatory infiltrate:
Folliculitis (acne) keloidalis
Folliculitis (acne) necrotica
Erosive pustular dermatosis
Nonspecific
|
Infections:
Bacterial, viral, fungal
Immunological:
Sarcoidosis, necrobiosis lipoidica, morphea, graft versus host disease.
Neoplasms:
Alopecia mucinosa, lymphoproliferative
Exogenous:
Burns and surgical and traumatic scars.
Dermatoses:
Psoriasis, immunobullous disorders
Hamartomas:
Organoid nevus
Miscellaneous
|
Abbreviations: CA, cicatricial alopecia; CCCA, central centrifugal cicatricial alopecia;
CCLE, chronic cutaneous lupus erythematosus; LPP, lichen planopilaris
In secondary cicatricial alopecia (SCA), the hair follicles are secondarily damaged
as a result of destructive processes within the skin.[5] The causes of SCA are elaborated in [Table 1].
Another classification categorizes CA into “stable” and “unstable” type[8]. “Stable” CAs are secondary to isolated events that cause permanent scarring in
a hair-bearing region. Whereas “unstable” cicatricial alopecias (UCAs) are secondary
to disorders that tend to progress and recur intermittently over the course of time.
Pathogenesis
The pathogenesis of PCA revolves mainly around the destruction of slow-cycling, pluripotent
hair follicle stem cells (HFSCs).[9]
[10] These HFSCs are located in the “bulge” region of a hair follicle in the outer root
sheath (i.e., at the site of attachment of the arrector pili muscle to the outer root
sheath). The newer insights into the pathogenesis of PCA mainly involves the HFSCs'
destruction theories, impairment of self-maintenance of HFSCs, alteration of lipid
metabolism, neurogenic inflammation theory, and environment and genetic factors.[4]
[11]
[12]
[13]
[14]
[15]
[16]
[17]
[18]
[19]
[20]
[21]
[22]
[23]
[24]
[25]
[26]
[27]
[28]
[29]
[30] Although various treatment modalities are presently based on immune-modulation,
none is definite or completely curative.
In contrast, as the pathogenesis of SCA is already known (due to trauma, infection
or others), various surgical modalities are present to give definite results in case
of SCA.[31]
[32]
[33]
[34]
[35] There is always a tell-tale sign in cases of secondary cases like burns in other
areas, visible sutured, scarred zone, history of previous infection, or radiation
therapy postcancer treatment.
Clinical Evaluation in CA
Clinical Evaluation in CA
Primary CA presents a therapeutic challenge. During the initial phase of the disease,
process symptoms and signs might mimic androgenetic alopecia (AGA). In the later stages
of CA, the clinical picture looks similar in all the subtypes of PCA. A proper history,
clinical examination, trichoscopic evaluation, and scalp biopsy helps in arriving
at appropriate diagnosis. Majority of PCA occur in early adult life, with the exception
of the two genetic conditions, keratosis follicularis spinulosa decalvans and Marie
Unna hypotrichosis, which are seen in childhood.[36] Frontal fibrosing alopecia (FFA) occurs in the postmenopausal age group. Central
centrifugal cicatricial alopecia (CCCA), acne keloidalis, and dissecting cellulitis
of scalp are seen in people of African origin.[37] Patients with PCA usually present with associated symptoms of scalp scaling, pigmentation,
itching, irritation, pustules, swelling on scalp, active hair loss in patches or a
diffuse loss, hair loss in other regions of the body apart from the scalp, and pain
on the scalp. These symptoms when present help the physician to think of PCA while
evaluating a patient with hair loss.[38] Location of the lesions provides the clue to the diagnosis of PCA.[38] Vertex lesions are seen in CCCA and dissecting cellulitis of scalp. Frontal involvement
is seen in FFA. Acne keloidalis is seen in the nape of the neck.
Chronic cutaneous lupus erythematosus (CCLE) presents with maximal clinical activity
in the center. Perifollicular inflammation, follicular plugging, atrophy, and dyspigmentation
are the characteristic findings.[38] In lichen planopilaris (LPP), the clinical activity is seen in periphery of the
lesions, with features of perifollicular erythematous and violaceous papules, spinous
follicular hyperkeratosis and multifocal disease.[38] Associated findings of oral cavity, skin and nails characteristic of lichen planus
may be observed in cases of LPP.[1] FFA presents with a band-like alopecia over the frontal region of the postmenopausal
women. Involvement of eyebrow hair may be observed in FFA.[38] Diagnosing the subtypes of long-standing PCA clinically poses a challenge, since
atrophic scarring is seen in all the conditions of PCA, except folliculitis decalvans
which present s with hypertrophic scarring.[38] Features suggestive of dissecting cellulitis of scalp are pustules, honey-colored
crusts at the periphery of the lesions, and tufting of the hair.[38] In dissecting cellulitis of scalp, the features observed are purulent discharge,
boggy scalp, and sinus tracts.[38]
Diagnosis of CA is made by correlating clinical, trichoscopic, and scalp biopsy findings.
Scalp biopsy should be done on the active margins of the CA lesions.[39] Two biopsy specimens are recommended for vertical and horizontal sections to analyze
the involvement of various structures of the hair follicle. Hair pull test performed
at the margins of the CA lesions indicate the activity of the disease. A positive
hair pull test denotes active disease.
Management
PCA throws up a diagnostic and therapeutic dilemma to the treating surgeon. The aim
of treatment currently focuses on reducing symptoms and reducing or stopping the progression
of the disease. The mainstay of treatment of LMPCA is immunosuppression and of NMPCA
is antimicrobials or dapsone.[40] In cases of PCA, surgical treatment can be planned only after complete disease stability,
which is sometimes never achieved. Chloe et al in a systematic review of hair transplant
in PCA found a 76% positive outcome rate.[41] Eight out of 34 patients experienced negative results, including those with a diagnosis
of LPP or FFA. FFA had the worst results.
In cases of secondary scarring alopecia, we have various options including hair transplantation,
scalp reduction surgeries, tissue expansion and flap surgeries.[8]
[42]
[43] There are various limitations in case of secondary alopecias due to the excessive
scarring, which can occur in burns and postradiation patients. Umar described successful
results in three cases of secondary alopecias using non-scalp donor sites.[44]
Challenges Faced in Hair Transplant (HT) for CA due to the Following Factors:
Challenges Faced in Hair Transplant (HT) for CA due to the Following Factors:
-
The question pertaining to when to opt for the surgical treatment remains unanswered.
The common consensus is that a quiescent disease phase of 2 years is necessary before
the surgical treatment.[8] The difficulty in documenting the disease inactivity poses a further dilemma to
the surgeon.
-
The vascularity of the scar tissue is unpredictable and varies in terms of interpatient
and intrapatient variables.
-
Graft survival in scar tissue is unpredictable.
-
There is always a risk of disease relapse and loss of grafted follicular units (FUs).
-
In extensive scarring, donor insufficiency may limit the possibility of HT.
-
Multiple sessions are required for a good cosmetic outcome.
-
Possibility of future evolution of male pattern baldness (MPB)/female pattern hair
loss (FPHL) should be considered in every CA undergoing HT around the areas treated.
-
The surgical procedure in itself might trigger the relapse of CA, which is reported
in cases of LPP.
-
Wound healing characteristics vary widely and remain unpredictable.
-
Common complications encountered after HT are hypertrophic scars, graft rejection,
infection, and corkscrew hair.
Factors to Be Considered before Planning HT in CA
Factors to Be Considered before Planning HT in CA
Donor-Recipient Area Mismatch
Estimation of the donor-recipient area remains the most critical decision in choosing
HT for CA. In the majority of the patients, the available donor FUs are insufficient
to treat the existing as well as the probable future areas of CA. This is more so
in those patients with CA involving the donor area. Body hair harvesting should be
considered in all patients to optimize the donor reserves.
Scar Texture
In atrophic scar tissue, the recipient sites depth may be shallow and not be sufficient
to accommodate the entire implanted FU due to lack of soft tissues; moreover, there
is risk of insufficient perfusion to the implanted FU. In hypertrophic scars, the
implanted follicles might not reach adequate depth to take up the blood supply from
the subcutaneous tissue due to the thickness of scar tissue. A CA scar can have atrophic
and hypertrophic components in the same lesion. History of hypertrophic scar/keloid
over the previous surgical sites indicates a poor prognosis and requires careful planning.
Location of the Scar
Scars involving the hair line requires careful planning with a relatively high-density
FU (25–35 FU/cm), since these regions have good adjacent vascularity. Shao et al used
a follicular density of 20 to 48 FU/cm2 in his series.[45] FU placed on the normal-looking skin adjacent to the scars provides good camouflage.
Vertical scars are difficult to conceal than the horizontal or oblique scar, since
vertical scars need long hair to fall over the alopecia in order to provide cover.
Scar Vascularity
Grafts at the center of a large scar are distant from a good vascular supply, and
ischemic injury may lead to necrosis and infection in addition to poor growth of the
FU. Vascularity might not be uniform all over the scar tissue.
Alopecia Reduction Versus HT
HT is preferable to scar excision/alopecia reduction (AR) in patients with minimal
scalp laxity, those with scalp where there are limitations in surgical undermining
of the tissue due to lack of stretch of scalp intraoperatively, poor donor reserves
(Hamilton Norwood scale 7), very young patients with evolving hair loss, and those
with unrealistic expectations.[46] Good candidates for AR are those 40 years and above with stable hair loss pattern
(Hamilton–Norwood scale 4, 5, 6), good donor hair in the occipital region, smaller/solitary
CA lesion, and those who accept hair loss on the incision lines.[46] Scalp flaps can be considered in cases of CA, where local flaps can be utilized
without having a secondary defect to restore.[47]
Techniques to Be Modified to Improve the Cosmetic Outcome of HT in CA
Techniques to Be Modified to Improve the Cosmetic Outcome of HT in CA
Assessing Vascularity of the Scar Tissue
Adrenaline usage should be avoided in the recipient site, as vascularity is already
compromised due to scarring. To test the blood supply of a large area, it is recommended
that a portion of the recipient area is anaesthetized with a 2% lidocaine solution
without epinephrine.[8] A 19-G needle can be used to make several incisions on the recipient scar tissue.
The evidence of bleeding during the incisions with the needle denotes the presence
of adequate vascularity in the tissue. The absence of bleeding points indicates compromised
blood supply, and the options of other surgical modalities should be explored. The
vascularity of the scar tissue should be periodically assessed intraoperatively within
the same scar tissue by evaluating the bleeding while making the recipient area incisions.
FU Graft Size Selection
Follicular family ([FF]—two closely placed FUs) are useful in creating an illusion
of density in a scarred area. FF grafts provide the option of implanting a greater
number of hair follicles within the same recipient site and reduce the need to make
multiple incisions, minimizing the vascular damage.[8]
Recipient Area Slit Making for Implantation of FU Grafts
Recipient sites should be smaller in tissue with great laxity or very little recoil
to prevent the popping of grafts, whereas sites need to be larger if the scar has
limited laxity or significant recoil to prevent excessive manipulation of grafts during
insertion.[8] To minimize the vascular damage in the recipient area, it is recommended to make
the recipient site incisions parallel to the direction of hair growth.[8] While making the recipient site incisions parallel to the existing hair follicles,
acute angle of incision leads to increased depth at the level of epidermis compared
with less acute angle of incision in spite of using the same size needle or blade.
In recipient sites requiring acute angle incisions (eyebrow, temporal regions, and
the hairline in supra-auricular and preauricular regions), the diameter of the needle/size
of the recipient slits should be reduced to prevent wider and deeper implantation
slits over the scarred tissue.[8] Atrophic scars are too shallow to accommodate and adequately perfuse the FU. Hence,
the recipient incision has to be made deeper by creating an incision at a more acute
angle. In hypertrophic scars, the recipient sites have to be made deeper than usual
to improve the perfusion to the FU.
Test Grafting and Density of FU in Recipient Area
Patients with CA can be subjected to a test graft session and wait time of 6 months
to assess the outcome before venturing into a full procedure.[48] The density of FU per cm is the most difficult decision a surgeon needs to make
while doing HT in CA. The standard recommendation is not to perform higher density
FU grafting in CA. In CA regions with poor blood supply, FU densities of 15 to 20
FU/cm2 are considered optimal for better survival of the FU. In CA lesions with good blood
supply, the density of FU can be 20 to 30 FU/cm2.[8] To provide optimal density of hairs for a better coverage, the CA lesions can be
grafted in a staged manner; a second surgery 9 to 12 months after first surgery is
recommended to optimize the FU survival. Scalp tissue tends to be thicker in atrophic
regions of CA, and vascularity increases following first HT surgery.[49]
Adjuvant Treatment
Topical minoxidil (2–5%) application in the recipient area for 2 weeks before and
at least 5 weeks after surgery increases the local blood supply, prolongs anagen (hair
growth) phase, and leads to subsequent improvement in graft survival.[49]
Pentoxifylline, 400 mg, thrice a day with meals, for 2 weeks prior to surgery has
been advised to increase the oxygenation in the recipient area.[50]
Intralesional steroid injection in a stepwise manner into the scar tissue prior to
HT has been tried with good outcome, in order to reduce the thickness of the scar
and increase the pliability.[49]
Prior treatment of the scar tissue with fractional co2 lasers have been reported to
be beneficial in improving the outcome of FU grafting in CA. The accelerated growth
of transplanted hairs has been reported due to the promotion of neoangiogenesis and
the inductions of various growth factors and cytokines during the wound healing process
after laser-induced thermal damage. A hole-to-hole interval of 5 mm is assumed to
be suitable for laser pretreatment of the recipient area in CA.[51]
Platelet-rich plasma (PRP) has been tried with good success as an adjuvant in CA to
improve the outcome of HT. PRP has been tried prior to the surgery, during the surgery
on the recipient area sites, and postsurgery management. PRP influences wound healing
and scar remodeling and may provide better FU survival.[52]
Additional treatments like topical steroids/dapsone/other immunosuppressants may need
to be continued post-HT, especially in PCA, to prevent disease progression/relapse.
Conclusion
HT is a definite option for CA, more so in secondary CA as compared with primary CA.
In primary CA, a waiting period of 2 to 5 years should be observed for complete disease
stability before undertaking HT. A proper counselling should be given about the possibilities
of negative results, especially in FFA and LPP, chances of disease relapse, or reactivation
after HT. Emphasis on continuing the medical management should be provided even after
HT in cases of primary CA.
