Viktor Migunov¹

¹Vilnius University, Faculty of Medicine

 Abstract

Background. Lichen planopilaris is an immune-mediated scarring alopecia. The disease usually manifests as pruritic or painful multifocal patches of alopecia with perifollicular erythema and follicular hyperkeratosis. The course of lichen planopilaris is usually unpredictable, and currently available treatment options do not lead to hair regrowth, unless the treatment is started at the very beginning stages of the disease.

Case report. In this case, a 35-year old female was diagnosed with lichen planopilaris. At the beginning of the treatment with topical steroids, clinical improvement was observed, and after that, sudden clinical deterioration followed. This clinical case shows the importance of systemic treatment at the very beginning stages of lichen planopilaris.

Discussion. The treatment of lichen planopilaris is challenging because of relapsing-progressive pattern of the disease. At this moment, there is no standardized therapeutic approach for this disease and treatment often relies on the physician’s personal experience, although there are some proposed therapeutic strategies.

Keywords. Lichen planopilaris, scarring alopecia, treatment.

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Medical Sciences 2021 Vol. 10 (1), p. 157-165, https://doi.org/10.53453/ms.2022.03.18

Lichen planopilaris: a case report

Viktor Migunov

1

1

Vilnius University, Faculty of Medicine

Abstract

Background. Lichen planopilaris is an immune-mediated scarring alopecia. The disease usually manifests as

pruritic or painful multifocal patches of alopecia with perifollicular erythema and follicular hyperkeratosis. The

course of lichen planopilaris is usually unpredictable, and currently available treatment options do not lead to hair

regrowth, unless the treatment is started at the very beginning stages of the disease.

Case report. In this case, a 35-year old female was diagnosed with lichen planopilaris. At the beginning of the

treatment with topical steroids, clinical improvement was observed, and after that, sudden clinical deterioration

followed. This clinical case shows the importance of systemic treatment at the very beginning stages of lichen

planopilaris.

Discussion. The treatment of lichen planopilaris is challenging because of relapsing-progressive pattern of the

disease. At this moment, there is no standardized therapeutic approach for this disease and treatment often relies

on the physician’s personal experience, although there are some proposed therapeutic strategies.

Keywords. Lichen planopilaris, scarring alopecia, treatment.

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Introduction

Lichen planopilaris (LPP) is a primary lymphocytic-

mediated cicatricial alopecia . It’s a rare disease of

the scalp and it’s the cause of alopecia in about

1.25% of patients and the cause of scarring alopecia

in up to 25% (1). In LPP, hair follicles are selectively

destroyed by a chronic lymphocytic inflammatory

process that often results in irreversible scarring

alopecia if not treated (2). LPP is usually seen

between 40 and 60 years and is more common in

Caucasian women (3-5). The disease presents as

pruritic or painful multifocal patches of alopecia

with perifollicular erythema and follicular

hyperkeratosis (6, 7). LPP patches are typically

distributed over the central scalp, but the disease

may also affect non-scalp areas, including

eyebrows, eyelashes and axillae (5, 8). Based on the

clinical distribution of the lesions, LPP has three

clinical variations: classic form, frontal fibrosing

alopecia (FFA) and Graham-Little-Piccardi-

Lassueur (GLPL) syndrome. The classic form is the

most common and usually involves the vertex and

the parietal part of the scalp (3, 9). GLPL syndrome

is characterized by the triad: scarring alopecia of the

scalp, non-scarring alopecia in the armpit and pubis

and lichenoid papules on the trunk and extremities

(10). FFA is characterized by a progressive

recession of the fronto-temporal hairline associated

with a loss of eyebrows, eyelashes and peripheral

body hair, affecting mainly postmenopausal women

(11-13). The diagnosis of LPP is based on physical

assessment, along with dermoscopic and

histological examination.

Case report

A 35-years-old female went to a dermatologist with

complaints of increased hair shedding and sensitive

scalp skin which was noticed 3-years ago after

withdrawal of contraception. The patient used hair

loss decreasing shampoos and serums, also had

mesotherapy and 6 platelet-rich plasma (PRP)

procedures. The patient was previously diagnosed

with polycystic ovary syndrome, autoimmune

thyroiditis and currently taking L-Thyroxine.

Trichological evaluation revealed diffuse thinning at

the vertex region of the scalp (Fig. 1) Digital

dermoscopy showed varying hair shaft thickness,

perifollicular erythema and follicular

miniaturization (Fig. 2) In suspicion of scaring

alopecia, scalp punch biopsy was performed and

histological examination showed follicular

hyperkeratosis, perivascular lymphocytic

inflammation, reduction of arrector pili muscle and

reduction of sebaceous glands which are typical

findings of LPP.

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Fig.1 Diffuse thinning of the hair at the vertex region.

Fig. 2 Trichoscopy of the patient, revealing perifollicular erythema and follicular hyperkeratosis.

The patient was prescribed with topical 5%

Minoxidil and Clobetasol solution also Doxycycline

100mg/day. After two months of treatment clinical

improvement was observed, hair shedding

decreased, pruritus was absent. Antinuclear

antibody (ANA) test performed to exclude systemic

lupus erythematosus and the result was negative.

The patient continued to use prescribed medications.

Five months later the patient came for a follow-up

visit. Trichological evaluation revealed diffuse

thinning at the vertex region of the scalp. Digital

dermoscopy showed varying hair shaft thickness,

follicular miniaturization, telangiectasias and

increased perifollicular erythema which indicate an

exacerbation of the disease. (Fig. 3) Methotrexate

treatment was considered but the patient refused it

and continued previous treatment.

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Fig. 3 Trichoscopy of the patient, revealing increased perifollicular erythema, follicular hyperkeratosis and

telangiectasias.

Four months later the patient came for a follow-up visit. The patient complained of pruritus and scaling on the

scalp skin. Trichological evaluation (Fig.4) revealed erythema in the occipital region, dermoscopy showed

telangiectasias and yellowish scabs (Fig. 5) The patient received Triamcinolone solution 40mg/ml injection 0.5ml

to the occipital scalp region and continued treatment with Doxycycline 100mg/day, 5% Minoxidil and Clobetasol

solution.

Fig. 4 Erythema at the occipital region.

Fig. 5 Telangiectasias and yellowish scabs

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One month later the patient came for a follow-up visit. Trichological evaluation (Fig.6) revealed a 1

´

1 cm

thinning hair spot, erythema and scaling in the occipital region. The patient received Triamcinolone solution

40mg/ml injection 0.5ml. Due to clinical exacerbation Hydroxychloroquine (HCQ) 200mg/day as a systemic

therapy was added to a previous treatment.

Fig. 6 Thinning hair spot on the vertex

One month later the patient came for a follow-up visit. Significant clinical deterioration was observed.

Trichological evaluation (Fig. 7) revealed new balding areas, erythema decreased. Complete blood count, C-

reactive protein, Aspartate Aminotransferase, Alanine Aminotransferase, Gamma-Glutamyl Transpeptidase,

creatinine and urea tests were performed due to higher 400mg HCQ dose prescription. All tests were within a

normal range, HBV, HBC, HIV and Quantiferon tests were negative. HCQ dose was increased to 400mg/day and

0.1% Tacrolimus ointment was added to the previous treatment.

Fig. 7 New lesions at the vertex

Five months later the patient came for a follow-up visit and significant clinical improvement was observed. Hair

shedding and erythema decreased, new hairs are regrowing in the lesion area. (Fig. 8)

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Fig. 8 Regrowing hairs in the lesion area.

Discussion

Treatment of LPP is challenging because of the

relapsing-progressive pattern of the disease (2).

According to one study, 42.9% of LPP patients

required third-line medications, and 11.3% could

not achieve complete response despite using

multiple third-line drugs (14). The primary

treatment goals of LPP are to reduce symptoms and

to stop disease activity, hence preventing the

development of further alopecic regions (2, 15). For

this moment there is no standardized therapeutic

approach for this disease and LPP treatment often

relies on the physician’s personal experience,

although there are some proposed therapeutic

strategies (Fig.9).

Topical corticosteroids (TCS) are commonly used as

a first-line treatment and they can be used either in

monotherapy or in combination with other topical or

systemic therapies (16, 17).

The existing studies showed a success rate of 53.9%

in patients treated with TCS, while a response rate of

56.7% was observed with intralesional

corticosteroids (ILCS) (18). Oral steroids should be

considered only in rapidly progressive cases because

of high relapse rate of 80% within 1 year after drug

withdrawal (17). Hydroxychloroquine (HCQ)

suggested as initial systemic therapy (400mg/d) and

several studies investigating its efficacy showed

51.2% response rate, while other studies showed

higher success rates, with figures ranging from 40%

to 76% (18, 19). If manifestations of the disease

continue after 2 months of treatment, methotrexate

(MTX) should be considered as a second-line

therapy. The studies showed efficacy of MTX with

response rate of 87.5% (17). Mycophenolate mofetil

(MMF) and cyclosporine (CSP) are third-line

therapy drugs with the response rates of 48.5% and

77.3% (2). Although CSP has much higher response

rate, MMF is preferred for a long time period,

because CSP shows high relapse rate of 80% and

serious side effects in a long-term use (2, 16). In one

study Pioglitazone (15mg/day) showed a good result

with response rate of 66.2%, while other studies

showed less positive outcomes with a response rate

of 31.8% (20, 21). Pioglitazone (15mg/day for 8

months) had a positive outcomes in managing

multiresistant LPP cases (22).

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Classic lichen planopilaris

Limited forms Extensive forms

Topical/intralesional/potent steroids Hydroxychloroquine First-line

Methotrexate Second-line

Cyclosporine

Mycophenolate mofetile Third-line

Pioglitazone

Fig. 9 A proposed treatment strategy for classic lichen planopilaris (19).

Conclusions

• Treatment of lichen planopilaris is

challenging and unpredictable because of

relapsing-progressive pattern of the

disease.

• Patients tend to refuse systemic drugs

which can lead to poor treatment outcomes.

• Systemic treatment should be considered at

the very beginning stages of the lichen

planopilaris.

• Treatment results reported in the literature

are variable, thus treatment relies on

physician’s experience and patient’s

condition.

• Intralesional corticosteroids don’t have a

significant advantage over topical steroids.

• Hydroxychloroquine 400mg/day should be

considered as initial systemic treatment.

• Mycophenolate mofetil should be

considered as an alternative to cyclosporine

for long-term treatment.

• Pioglitazone should be considered for

managing multi-drug resistant LPP cases.

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