Alopecias

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© Springer Nature Switzerland AG 2020
A. Tosti et al. (eds.)Hair and Scalp Treatmentshttps://doi.org/10.1007/978-3-030-21555-2_11



11. Scarring Alopecias



Sergio Vañó-Galván1  , David Saceda-Corralo2 and Rodrigo Pirmez3


(1)
Ramon y Cajal Hospital, Trichology Unit, Dermatology Department, Madrid, Spain

(2)
Ramón y Cajal University Hospital, Department of Dermatology, Madrid, Spain

(3)
Department of Dermatology Santa Casa da Misericordia, Rio De Janeiro, Brazil

 



 

Sergio Vañó-Galván


Keywords

Cicatricial alopeciaFrontal fibrosing alopeciaLichen planopilarisFolliculitis decalvansDiscoid lupusFibrosing alopecia in a pattern distributionDissecting cellulitisAcne keloidalis nuchaeCentral centrifugal cicatricial alopeciaScarring alopecia


Introduction


Hair loss has been demonstrated to cause a negative impact on self-confidence and self-esteem and can lead to higher levels of anxiety and depression and social implications. Patients with scarring alopecias in particular suffer a substantial impairment of quality of life and high levels of distress [1].


Scarring or cicatricial alopecias are a heterogeneous group of disorders characterized by the irreversible destruction of hair follicles. Follicular damage in scarring alopecias may be either primary or secondary. In primary cicatricial alopecia (PCA), the hair follicle itself is the principal target for destruction. Secondary cicatricial alopecias result from events not directed to the follicular unit that end up having an effect on and eventually destroying the follicle. The permanent follicular scarring in these cases is the result of the close proximity of the follicles to the primary pathological process [2].


Clinically, the hallmark of cicatricial alopecias is the loss of follicular openings, while under pathology, they are characterized by the replacement of follicular structures with fibrous tissue [3].


In 2001, the North American Hair Research Society proposed a classification of PCA based on the predominant inflammatory infiltrate found on scalp biopsy (Table 11.1) [4]. Even though arguments may be raised against this classification, it offers a reasonable and practical approach to this group of disorders, and therefore it is adopted in this chapter.


Table 11.1

NAHRS classification for primary alopecias












































Lymphocytic


Chronic cutaneous lupus erythematosus


Lichen planopilaris (LPP)


 Classic LPP


 Frontal fibrosing alopecia


 Graham Little syndrome


 Fibrosing alopecia in a pattern distribution (FAPD)a


Classic pseudopelade (Brocq)


Central centrifugal cicatricial alopecia


Alopecia mucinosa


Keratosis follicularis spinulosa decalvans


Neutrophilic


Folliculitis decalvans


Dissecting cellulitis/folliculitis


Mixed


Folliculitis (acne) keloidalis nuchae


Folliculitis (acne) necrotica


Erosive pustular dermatosis


Nonspecific



aFAPD has been included here, despite not being present in the original classification


Recent data from a multicenter study involving 22 institutions specialized in hair disorders revealed that frontal fibrosing alopecia (FFA) is now the most frequent type of PCA in this setting, followed by lichen planopilaris (LPP), folliculitis decalvans (FD), discoid lupus (DLE), and fibrosing alopecia in a pattern distribution (FAPD) [5].


Treatment of PCA is many times challenging, and there are no fully satisfactory evidence-based regimens available for the treatment of such conditions. Here, we will focus on the treatment of the main causes of PCA according to published data and our experience on the field. In scarring conditions, the central aim of treatment will not be hair regrowth but rather to reduce symptoms and, most importantly, to stop disease progression. Early treatment is key to minimizing the extent of permanent alopecia.


Lichen Planopilaris


Lichen planopilaris (LPP) is a primary scarring alopecia in which a lymphocytic inflammatory infiltrate occurs at the isthmus level and destroys the follicular stem cells of the bulge. It mainly affects women, although it may appear also in men [6].


Its etiopathogenesis has not been clarified. PPAR-gamma deletion in hair follicle stem cells causes a similar inflammatory reaction that leads to epithelial-mesenchymal stem cell transition and fibrosis [7, 8].


LPP may appear at any part of the scalp, but it usually affects the vertex. The typical clinical presentation consists of small patchy hair loss in a diffuse distribution with perifollicular inflammation. The patches of alopecia may coalesce leaving large areas of scarring. It frequently associates pruritus and scalp dysesthesia that may be severe [6, 9].


Trichoscopy shows loss of follicular openings and small tufting with perifollicular erythema and scaling, and it helps to assess the response to therapy (Fig. 11.1).

../images/440271_1_En_11_Chapter/440271_1_En_11_Fig1_HTML.jpg

Fig. 11.1

Trichoscopy of lichen planopilaris before and after systemic treatment


Histology shows a lichenoid infiltrate targeting the infundibulum and isthmus, with lamellar concentric fibrosis. Sebaceous glands are destroyed at early stages.


Management


Topical corticosteroids are recommended to control mild inflammation, and intralesional steroids can be added regularly (Table 11.2). If inflammation increases or symptoms appear, one or more oral therapies should be started, such as hydroxychloroquine, doxycycline, and retinoids [10, 11]. For baseline and follow-up recommendations and possible side effects regarding the use of hydroxychloroquine refer to Tables 11.10 and 11.11, at the end of chapter. For acute inflammation or presence of severe symptoms, systemic treatment with corticosteroids (prednisone, dexamethasone) or immunosuppressive drugs (cyclosporine, mycophenolate, methotrexate (15-25mg/weekly)) is recommended [12, 13]. Other therapies such as low-dose oral naltrexone, low-level laser therapy, oral pioglitazone, and platelet-rich plasma injections have been proposed to control symptoms and progression of the alopecia.


Table 11.2

Proposal of a therapeutic approach in lichen planopilaris










































































 

Treatment

Dosage

Duration

Mild inflammation (mild follicular erythema and hyperkeratosis)


No symptoms

Topical corticosteroid

Four to seven times per week

Treatment can be stopped and check for relapses


Mild pruritus

Topical corticosteroid

Four to seven times per week

Until improvement


Consider intralesional steroids (triamcinolone acetonide)

4–8 mg/mL every 8–12 weeks

Until improvement


Moderate inflammation (moderate hyperkeratosis or moderate pruritus)


Maintain previous recommendations with topical and intralesional corticosteroids until improvement


Add one or more of the following oral therapies

Hydroxychloroquine

200 mg/12–24 h (5 mg/kg/day)

12 months


Doxycycline

50–100 mg daily

3 months


Oral retinoids

Isotretinoin 0.5–1 mg/kg daily

6–12 months


Acitretin 25–50 mg daily

6–12 months


Severe inflammation (severe hyperkeratosis and erythema/severe pruritus/pain)


Maintain previous recommendations with topical and intralesional corticosteroids until improvement


Choose one of the following therapies

Oral cyclosporine

3.5–5 mg/kg/day

4–6 months until tapering


Systemic steroids

Prednisone 1 mg/kg/day

1–2 months until tapering


Dexamethasone 0.1 mg/kg twice a week

3–4 months until tapering


Oral mycophenolate mofetil

1 g twice a day

4–6 months until tapering


After control of the inflammatory flare, keep one of the previous options for moderate inflammation


If bad control of the disease, consider adding oral naltrexone (3 mg daily), oral pioglitazone (15 mg daily), low-level laser therapy (655 nm, 15 minutes daily), or platelet-rich plasma injections


The Lichen Planopilaris Activity Index (LPPAI) is a clinical scale to monitor response to therapy in LPP. It includes symptoms (pain, pruritus, burning), clinical inflammation (erythema, scaling), pull test, and progression of the disease [10].


Frontal Fibrosing Alopecia


Frontal fibrosing alopecia (FFA) is a primary scarring alopecia with an increasing incidence in the last decade, probably being the most frequent alopecia nowadays [5]. It occurs mainly in postmenopausal women, but it also appears in young women and men [14].


The cause behind this alopecia is unclear. Hormonal imbalance in genetically predisposed individuals could trigger an autoimmune reaction against the pilosebaceous unit. The inflammatory infiltrate could induce epithelial–mesenchymal stem cell transition and fibrosis as in lichen planopilaris.


Clinically, a progressive recession of the frontotemporal hairline is observed. It associates local inflammation with perifollicular erythema and scaling , accompanied by itch or burning occasionally. Areas of scarring hair loss on the scalp are pale and show local atrophy with depression of the frontal veins . Total or partial loss of the eyebrows is common, as well as loss of body hair. Three main clinical patterns of progression of the disease have been described: linear (Fig. 11.2), diffuse (worst prognosis), and pseudo-fringe pattern (best prognosis) [15]. Trichoscopy and biopsy show similar features as those observed in lichen planopilaris.

../images/440271_1_En_11_Chapter/440271_1_En_11_Fig2_HTML.jpg

Fig. 11.2

Patient with frontal fibrosing alopecia in a linear pattern


There are other clinical findings of FFA on facial skin, such as facial papules, diffuse erythema, glabellar red dots, and lichen planus pigmentosus [16, 17]. Facial papules are small skin-colored papules that appear most frequently on the temples and the chins (Fig. 11.3). Although an original report described lichenoid inflammation of vellus follicles [18], recent evidence indicates that they are caused by enlargement of the sebaceous glands [19].

../images/440271_1_En_11_Chapter/440271_1_En_11_Fig3_HTML.jpg

Fig. 11.3

Facial papules in a frontal fibrosing alopecia patient


Management


Treatment (Table 11.3) aims to stop the progression of the frontotemporal recession. Local treatment consists of topical corticosteroids and topical calcineurin inhibitors. Frequency of application depends on the grade of inflammation [20]. According to current evidence, oral 5-alpha reductase inhibitors (dutasteride, finasteride) are the most effective systemic treatment to stop progression of the alopecia [21]. These medications, however, should be prescribed with caution in women with personal or familial history of breast cancer . Other oral therapies used in FFA include antimalarials (hydroxychloroquine), retinoids (isotretinoin, acitretin), and pioglitazone [22, 23]. To assess efficacy of the treatment, we recommend taking systematic measures of the frontotemporal hairline recession and using trichoscopy to measure peripilar inflammation at the follow-up visits every 3–6 months [24, 25]. If progression of the alopecia stops, oral treatment should be maintained for 2 years until discontinuing to assure clinical response.


Table 11.3

Therapeutic management in frontal fibrosing alopecia






























































 

Treatment

Dosage

Duration

Local treatment


Mild inflammation

Pimecrolimus 1% cream or tacrolimus 0.1% ointment


Compound tacrolimus in cetaphil lotion to obtain 0.03% concentration

One to two times per week. Daily if needed


Daily

Until improvement


Can be utilized for long-term maintenance


Moderate inflammation

Topical calcineurin inhibitor and clobetasol propionate 0.05% solution


Consider triamcinolone acetonide injections

Four to seven times per week


Four to seven times per week


4–8 mg/mL every 8–12 weeks

Until improvement


(short-term follow-up)


Severe inflammation

Clobetasol propionate 0.05% solution and triamcinolone acetonide injections

Four to seven times per week


4–8 mg/mL every 8–12 weeks

Until improvement (short-term follow-up)


Partial eyebrows loss: topical calcineurin inhibitor + prostaglandin agonist eyedrops +/− triamcinolone acetonide injections 1–2 mg/mL


Systemic treatment


First-line therapies

Oral 5-alpha reductase inhibitors

Dutasteride 0.5 mg three to seven times a week


Finasteride 2.5–5 mg daily

2 years


Second-line therapies

Hydroxychloroquine

200 mg once or twice a day (5 mg/kg/day)

1–2 years


Oral retinoids

Isotretinoin 20 mg daily


Acitretin 20 mg daily

1–2 years


6–12 months


Third-line therapies

Pioglitazone

15 mg daily

6–12 months


Cosmetic approach


Facial papules: oral isotretinoin (5–30 mg daily for 2–12 months)


Complete loss of eyebrows: microblading or cosmetic tattooing


Hairline recession: partial hair prostheses or hair transplant (if 2 years without progression is confirmed)


Eyebrow loss leads to an important cosmetic impairment, so its treatment is mandatory. Intralesional injections of triamcinolone 1–2 mg/mL can be used for partial hair loss, and regrowth is possible in early stages. Long-term topical treatment with latanoprost /bimatoprost or travatoprost eyedrops and calcineurin inhibitors is also recommended. Microblading and cosmetic tattooing are encouraged when complete loss of the eyebrows.


Facial papules can be treated with low-dose oral isotretinoin, but relapses are common after discontinuing usage [26]. To improve cosmetic impairment on hairline recession, partial hair prostheses or hair transplantation can be done, if stop of the alopecia is confirmed after 2 years.


Fibrosing Alopecia in a Pattern Distribution


Fibrosing alopecia in a pattern distribution (FAPD) is a primary scarring alopecia that involves androgenetic hair loss areas. The cases reported reflect a slight higher prevalence in women, but it also occurs in men [27–29]. The etiopathogenesis of this scarring alopecia is unknown, but the combination of hair follicle miniaturization and lichenoid inflammatory infiltrate leads to a permanent alopecia [27, 30].


The hair loss pattern is similar to male- or female-patterned androgenetic alopecia (Fig. 11.4). The scalp shows loss of follicular openings, with perifollicular erythema and scaling. There is no multifocal patchy alopecia , like in lichen planopilaris. Coexistence with frontal fibrosing alopecia has been described [27]. Pruritus and pain may be associated with the disease. Trichoscopy is similar to lichen planopilaris, but miniaturization is a typical feature. Biopsy is highly recommended, especially in doubtful cases. It shows miniaturization and lichenoid infiltration.

../images/440271_1_En_11_Chapter/440271_1_En_11_Fig4_HTML.jpg

Fig. 11.4

Fibrosing alopecia in a pattern distribution in a 64-year-old female patient


Management


Therapies in combination are recommended (Table 11.4). Oral 5-alpha reductase inhibitors seem to be the best option to stop hair loss. Systemic antiandrogens (cyproterone, contraceptives) may also be useful in women. Topical corticosteroids can reduce perifollicular inflammation and symptoms [27, 30]. Other treatment options useful in lichen planopilaris (oral pioglitazone , low-lever laser therapy, platelet-rich plasma injections, etc.) may be considered in selected cases.


Table 11.4

Proposal of therapeutic approach for fibrosing alopecia in a patterned distribution









































 

Treatment

Dosage

Duration

Topical treatment


Mild inflammation

Clobetasol propionate 0.05% solution

Four to seven times per week

Until improvement (short-term follow-up)


Moderate inflammation and/or symptoms

Clobetasol propionate 0.05% solution


Consider triamcinolone acetonide injections

Four to seven times per week


4–8 mg/mL every 8–12 weeks

Until improvement (short-term follow-up)


Associate topical minoxidil 2%–5% once or twice daily


(better low-propylene glycol preparations)


Oral treatment


Finasteride

Men: 1 mg daily


Women: 2.5–5 mg daily

The same recommendations as those for androgenetic alopecia


Dutasteride

0.5 mg daily


Consider other antiandrogen options for women following recommendations for female androgenetic alopecia


Consider oral anti-inflammatory treatment (doxycycline, hydroxychloroquine, etc.) following recommendations for lichen planopilaris


Discoid Lupus Erythematosus


Alopecia is a well-known sign in chronic discoid lupus erythematosus (DLE). Approximately 60% of patients with DLE have patches of scarring alopecia on the scalp [31]. Association with systemic lupus erythematosus is not frequent, and only 5–15% of the patients will develop it [32].


Clinically, scalp DLE lesions present as well-defined erythematous scaly plaques of alopecia (Fig. 11.5). Progressively, lesions develop cutaneous atrophy, hyperpigmentation areas (in dark-skinned patients), and permanent hair loss. Symptoms , like pruritus or tenderness, may be associated [31]. Trichoscopy shows follicular red dots, follicular keratotic plugs, thick arborizing vessels, and blue-gray dots as the main features described (Fig. 11.6) [33]. On histopathology, a lichenoid infiltrate with basal vacuolization of the epidermis and follicular epithelium can be observed. Mucin deposit in the dermis is also characteristic. Direct immunofluorescence presents granular deposits of IgG and C3, but it can be also negative [34].

../images/440271_1_En_11_Chapter/440271_1_En_11_Fig5_HTML.jpg

Fig. 11.5

Patch of alopecia due to discoid lupus erythematosus


../images/440271_1_En_11_Chapter/440271_1_En_11_Fig6_HTML.jpg

Fig. 11.6

Trichoscopy of discoid lupus erythematosus lesion with follicular keratotic plugs


Management


At early stages, DLE lesions may have an excellent response to treatment, and hair regrowth is possible. At late stages, treatment must be adjusted to activity of the disease and risk of relapses (Table 11.5).


Table 11.5

Therapeutic approach for discoid lupus erythematosus




















































 

Treatment

Dosage

Duration

Local treatment


Acute lesions

Topical Clobetasol propionate 0.05%

Four to seven times per week

Until improvement (short-term follow-up)


And/or triamcinolone acetonide injections

4–12 mg/mL every 8–12 weeks


Chronic lesions and relapses prevention

Clobetasol propionate 0.05% solution

Once or twice a week

3–4 months


And/or topical calcineurin inhibitors

Every day

Adjust to relapses


Systemic treatment


First-line therapies

Antimalarials

Hydroxychloroquine 200 mg daily or twice a day (5 mg/kg/day)


Chloroquine 155 mg three times a day for 10 days and then 155 mg daily

6–12 months


Adjust to respond and consider long-term use according to relapses


Second-line therapies

Add quinacrine

100 mg daily


Third-line therapies

Methotrexate

15–25 mg weekly


Other therapeutic options: retinoids, mycophenolate, dapsone


Photoprotection (sunscreen, hats, sun avoidance)


Smoking cessation

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Mar 23, 2021 | Posted by in Dermatology | Comments Off on Alopecias

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