Therapeutic management of classic lichen planopilaris: a systematic review

Figures & data

Table 1 Summary of the reviewed studies regarding treatment options for classic lichen planopilaris

Study	Type of study	Number of patients	Level of evidence	Associated therapies	Posology	Therapy duration	Outcome
Hydroxychloroquine (highest level of evidence: II; total number of patients: 127; global response rate: 51.2% [65 of 127]; response rate in monotherapy: 51.0% [52 of 102])
Naeini et al^Citation8	Randomized clinical trial	14	II	None	400 mg/day	6 months	Significant LPPAI decrease at months 2 and 4, with only erythema showing significant improvement at month 6 compared with the baseline. Three withdrawn patients
Chiang et al^Citation10	Case series	29	IV	None	NA	12 months	4% (4 of 29) responders 72% (21 of 29) partial responders
Lyakhovitsky et al^Citation1	Case series	25	IV	None (three patients)	NA	Mean follow-up period: 15.1±3.6 months (all patients)	Partial improvement in inflammation in two cases
				Topical steroids (17 patients)	NA		Partial and complete improvement in inflammation in seven and three cases, respectively
				Intralesional steroids (two patients)	NA		Partial improvement in inflammation in one case
				Topical calcineurin inhibitors (one patient)	NA		Partial improvement in inflammation in one case
				Oral + topical steroids (two patients)	NA		Partial improvement in inflammation in one case
Spencer et al^Citation11	Case series	22	IV	None	6.5 mg/kg/day	6–12 months	Nine patients showed improvement
Assouly and Reigagne^Citation2	Case series	12	IV	None	400 mg/day	6 months	No success
Donati et al^Citation9	Case series	12	IV	None	400 mg/day	6 months	Eight patients worsened and three responded well to treatment. One patient lost to follow-up.
Mehregan et al^Citation12	Case series	9	IV	None	NA	NA	Two patients showed improvement
Mirmirani et al^Citation13	Case series	3	IV	Intralesional and topical corticosteroids	NA	NA	No effect
Mirmirani and Karnik^Citation14	Case report	1	V	None	200 mg twice daily	NA	No effect
Methotrexate (highest level of evidence: II; total number of patients: 16; global response rate: 87.5% [14 of 16]; response rate in monotherapy: 87.5% [14 of 16])
Naeini et al^Citation8	Randomized clinical trial	15	II	None	15 mg/week	6 months	Significant LPPAI decrease at months 2, 4 and 6, with significant improvement in all assessed variables at month 6 compared with the baseline. One withdrawn patient.
Spencer et al^Citation11	Case report	1	IV	None	10 mg/week	6 months	No improvement
Topical corticosteroids (highest level of evidence: IV; total number of patients: 128; global response rate: 53.9% [69 of 128]; response rate in monotherapy: 53.3% [49 of 92])
Lyakhovitsky et al^Citation1	Case series	70	IV	None (42 patients)	NA	Mean follow-up period: 15.1±3.6 months (all patients)	Partial and complete improvement in inflammation in five and three cases, respectively
				Hydroxychloroquine (17 patients)	NA		Partial and complete improvement in inflammation in seven and three cases, respectively
				Oral tetracyclines (eight patients)	NA		Partial improvement in inflammation in three cases
				Oral retinoids (three patients)	NA		Partial improvement in inflammation in one case
Mehregan et al^Citation12	Case series	20	IV	None	NA	NA	14 responders
Chieregato et al^Citation15	Case series	30	IV	None (27 patients)	Twice daily for 21 days, then once daily for 21 days, and finally every other day for 40 days	12 weeks	Good results in 20 patients and mild improvement in six patients
				Systemic cyclosporine (two patients)	5 mg/kg/day for 15 days, then 3 mg/kg/day	45 days	Improvement: clinical remission and partial regrowth
				Topical cyclosporine (one patient)	Twice daily for 20 days, then once daily	60 days	Clinical remission and even partial regrowth on perilesional skin
Horn et al^Citation16	Case series	2	IV	Intralesional triamcinolone acetonide	Twice daily	NA	Decrease in symptoms and altering of skin lesion progression
				None	NA	NA
Abbasi and Orlow^Citation17	Case report	1	V	Topical tacrolimus	NA	2 years	Symptom relief but no clinical improvement
Garcovich et al^Citation18	Case report	1	V	Cyclosporine	NA	7 months	Symptoms relief, but disease progression
Isaac and McNeely^Citation19	Case report	1	V	Intralesional corticosteroids, systemic corticosteroids, and griseofulvin	NA	NA	Symptom relief and disease stabilization
Jayasekera et al^Citation20	Case report	1	V	None	NA	NA	Improvement
Lane et al^Citation21	Case report	1	V	Cyclosporine	Thrice daily	NA	Symptom relief
Rosina et al^Citation22	Case report	1	V	None	NA	40 days	Symptom relief
Pioglitazone (highest level of evidence: IV; total number of patients: 65; global response rate: 66.2% [43 of 65]; response rate in monotherapy: 72.3% [34 of 47])
Baibergenova and Walsh^Citation25	Case series	24	IV	None	15 mg/day	NA	Five patients with remission and 12 experiencing some improvement
Spring et al^Citation27	Case series	18	IV	Topical steroids, systemic retinoids, systemic cyproterone acetate, topical minoxidil, finasteride, and/or mycophenolate mofetil	15 mg/day	6.1–9.25 months	Two remissions, seven patients experiencing clinical improvement, and nine failures
Mesinkovsk et al^Citation26	Case series	22	IV	None	15 mg/day	Median of 10.5 months	Marked improvement in 16 patients
Mirmirani and Karnik^Citation14	Case report	1	V	None	15 mg/day	8 months	Improvement
Mycophenolate mofetil (highest level of evidence: IV; total number of patients: 33; global response rate: 48.5% [16 of 33]; response rate in monotherapy: 48.5% [16 of/33])
Cho et al^Citation28	Retrospective study of open-label, single-center study	16	IV	None	0.5 g twice daily for 4 weeks, then 1 g twice daily for 20 weeks	Up to 1 year	Ten responders (five complete and five partial) and two treatment failures
Spencer et al^Citation11	Case series	10	IV	None	2–6 g/day	3–6 months	Three patients showed improvement
Assouly and Reigagne^Citation2	Case series	5	IV	None	2 g/day	2–8 months	Improvement in two patients
Tursen et al^Citation29	Case report	1	V	None for the first 2 months, then triamcinolone acetonide (1 mg/kg per month)	1 g/day for 2 months, then 500 mg/day for 4 months	6 months	Marked improvement
Mirmirani and Karnik^Citation14	Case report	1	V	None	NA	NA	No effect
Oral tetracyclines (level of evidence: IV; total number of patients: 30; global response rate: 27.6% [8 of 29]; response rate in monotherapy: 31.6% [6 of 19])
Spencer et al^Citation11	Case series	15	IV	None	200 mg/day (doxycycline)	3–6 months	Four patients showed improvement
Lyakhovitsky et al^Citation1	Case series	11	IV	None (one patient)	NA	Mean follow-up period: 15.1±3.6 months (all patients)	Partial improvement
				Topical steroids (eight patients)	NA		Partial improvement in two patients
				Intralesional steroid (one patient)	NA		No improvement
				Topical calcineurin inhibitors (one patient)	NA		No improvement
Mehregan et al^Citation12	Case report	1	IV	None	NA	NA	No improvement
Ferrara and Byrd^Citation24	Case report	1	V	None	NA	NA	Partial response
Mirmirani and Karnik^Citation14	Case report	1	V	None	NA	NA	No effect
Intralesional corticosteroids (highest level of evidence: IV; total number of patients: 30; global response rate: 56.7% [17 of 30]; response rate in monotherapy: 50.0% [13 of 24])
Lyakhovitsky et al^Citation1	Case series	18	IV	None (15 patients)	NA	Mean follow-up period: 15.1±3.6 months (all patients)	Partial and complete improvement in inflammation in ten cases and one case, respectively
				Hydroxychloroquine (two patients)	NA		Partial improvement in one patient and complete improvement in one patient
				Oral tetracycline (one patient)	NA		No improvement
Mehregan et al^Citation12	Case series	7	IV	None	NA	NA	No responders
Horn et al^Citation16	Case series	2	IV	Topical betamethasone dipropionate 0.05% lotion	10 mg/mL monthly + twice daily	NA	Decrease in symptoms and altering of skin lesion progression
Isaac and McNeely^Citation19	Case report	1	V	Clobetasol propionate + systemic corticosteroids and griseofulvin	10 mg/mL	NA	Symptom relief and disease stabilization
Muñoz-Pérez and Camacho^Citation23	Case report	1	V	None	NA	8-month follow-up	Improvement, but small areas of permanent alopecia remained
Ferrara and Byrd^Citation24	Case report	1	V	None	NA	NA	Partial response
Cyclosporine (highest level of evidence IV; total number of patients: 22; global response rate: 77.3% [17 of 22]; response rate in monotherapy: 72.2% [13 of 18])
Assouly and Reigagne^Citation2	Case series	13	IV	None	4–5 mg/kg/day	4 months	Ten patients showed clinical improvement
Mirmirani et al^Citation13	Case series	3	IV	None	3–5 mg/kg/day	3–5 month	Improvement
Mehregan et al^Citation12	Case series	2	IV	None	NA	NA	No improvement
Chieregato et al^Citation15	Case series	2	IV	Topical steroids	5 mg/kg/day for 15 days, then 3 mg/kg/day	45 days	Improvement
Garcovich et al^Citation18	Case report	1	V	Topical betamethasone	3 mg/kg/day	7 months	Improvement
Lane et al^Citation21	Case report	1	V	Betamethasone valerate 0.12% foam	3 mg/kg/day twice daily	NA	Partial improvement
Oral retinoids (highest level of evidence: IV; total number of patients: 13; global response rate: 23.1% [3 of 13]; response rate in monotherapy: 22.2% [2 of 9])
Assouly and Reigagne^Citation2	Case series	6	IV	None	25 mg/day	NA	No success
Lyakhovitsky et al^Citation1	Case series	4	IV	Topical corticosteroids (three patients)	NA	Mean follow-up period: 15.1±3.6 months (all patients)	Partial improvement (one patient)
				Topical calcineurin inhibitors (one patient)	NA		No improvement
Spencer et al^Citation11	Case series	3	IV	None	25 mg/day	3–6 months	Two patients showed improvement
Oral steroids (highest level of evidence: IV; total number of patients: 15; global response rate: 73.3% [11 of 15]; response rate in monotherapy: 71.4% [10 of 14])
Mehregan et al^Citation12	Case series	11	IV	None	30–40 mg/day for at least 3 months with gradual taper	NA	Improvement in nine patients
Lyakhovitsky et al^Citation1	Case report	1	IV	None	NA	NA	No effect
Isaac and McNeely^Citation19	Case report	1	V	Intralesional corticosteroids, clobetasol propionate, and griseofulvin	NA	4–6 weeks	Symptom relief and disease stabilization
Ferrara and Byrd^Citation24	Case report	1	V	None	NA	NA	Partial response
Mirmirani and Karnik^Citation14	Case report	1	V	None	NA	NA	No effect
Griseofulvin (highest level of evidence: IV; total number of patients: 12; global response rate: 41.7% [5 of 12]; response rate in monotherapy: 45.5% [5 of 11])
Mehregan et al^Citation12	Case series	10	IV	None	NA	NA	Five patients showed improvement
Isaac and McNeely^Citation19	Case report	1	V	Intralesional corticosteroids, systemic corticosteroids, and clobetasol propionate	1 g/day	NA	No improvement
Metin et al^Citation30	Case report	1	V	None	12.5 mg/kg/day	3 months	No benefit
Topical calcineurin inhibitors (highest level of evidence: IV; total number of patients: 12; global response rate: 23.1% [2 of 12]; response rate in monotherapy: 11.1% [1 of 9])
Lyakhovitsky et al^Citation1	Case series	10	IV	None (seven patients)	NA	Mean follow-up period: 15.1±3.6 months (all patients)	Partial improvement in inflammation in one case
				Hydroxychloroquine (one patient)	NA		Partial improvement in inflammation in one case
				Oral tetracyclines (one patient)	NA		No improvement
				Oral retinoids (one patient)	NA		No improvement
Abbasi and Orlow^Citation17	Case report	1	V	None	NA	2 years	Symptoms relief, but no clinical improvement
Almaani et al^Citation31	Case report	1	V	None	Once daily	6 months	Little effect
Thalidomide (highest level of evidence: IV; total number of patients: 9; global response rate: 11.1% [1 of 9]; response rate in monotherapy: 11.1% [1 of 9])
Jouanique et al^Citation32	Case series	4	IV	None	100 mg/day for 1 month, then 200 mg/day	6 months	No clinical improvement
Assouly and Reigagne^Citation2	Case series	4	IV	None	100 mg/day	6 months	No success
George and Hsu^Citation31	Case report	1	V	None	150 mg/day for 1 month, then tapered to 50 mg qhs for another month	2 months	Improvement
Laser therapy (highest level of evidence: IV; total number of patients: 13; global response rate: 23.1% [3 of 13]; response rate in monotherapy: 23.1% [3 of 13])
Vavricka et al^Citation33	Case report	13	IV	None	NA	NA	Improvement in three patients and no effect in the remaining ten subjects

Abbreviations: LPPAI, Lichen Planopilaris Activity Index; NA, not available

Figure 1 Proposed treatment strategy for classic lichen planopilaris.

Notes: *Topical/intralesional steroids may be added to systemic therapies in the case of persistence of limited active areas. ^§A short course of systemic steroids should be considered only to halt the progression and to improve symptoms in rapidly progressive and severe cases.

NaeiniFFSaberMAsilianAHosseiniSMClinical efficacy and safety of methotrexate versus hydroxychloroquine in preventing lichen planopilaris progress: a randomized clinical trialInt J Prev Med201783728584619

 PubMed Web of Science ®Google Scholar

ChiangCSahDChoBKOchoaBEPriceVHHydroxychloroquine and lichen planopilaris: efficacy and introduction of Lichen Planopilaris Activity Index scoring systemJ Am Acad Dermatol201062338739220061052

 PubMed Web of Science ®Google Scholar

LyakhovitskyAAmichaiBSizopoulouCBarzilaiAA case series of 46 patients with lichen planopilaris: demographics, clinical evaluation, and treatment experienceJ Dermatolog Treat201526327527924913130

 PubMed Web of Science ®Google Scholar

SpencerLAHawrylukEBEnglishJC3rdLichen planopilaris: retrospective study and stepwise therapeutic approachArch Dermatol2009145333333419289775

 PubMedGoogle Scholar

AssoulyPReigagnePLichen planopilaris: update on diagnosis and treatmentSemin Cutan Med Surg200928131019341936

 PubMed Web of Science ®Google Scholar

DonatiAAssoulyPMatardBJouaniqueCReygagnePClinical and photographic assessment of lichen planopilaris treatment efficacyJ Am Acad Dermatol201164359759821315955

 PubMed Web of Science ®Google Scholar

MehreganDAVan HaleHMMullerSALichen planopilaris: clinical and pathologic study of forty-five patientsJ Am Acad Dermatol1992276 Pt 19359421479098

 PubMed Web of Science ®Google Scholar

MirmiraniPWilleyAPriceVHShort course of oral cyclosporine in lichen planopilarisJ Am Acad Dermatol200349466767114512914

 PubMed Web of Science ®Google Scholar

MirmiraniPKarnikPLichen planopilaris treated with a peroxisome proliferator-activated receptor gamma agonistArch Dermatol2009145121363136620026843

 PubMed Web of Science ®Google Scholar

ChieregatoCZiniABarbaAMagnaniniMRosinaPLichen planopilaris: report of 30 cases and review of the literatureInt J Dermatol200342534234512755968

 PubMed Web of Science ®Google Scholar

HornRTJrGoetteDKOdomRBOlsonEGGuillMAImmunofluorescent findings and clinical overlap in two cases of follicular lichen planusJ Am Acad Dermatol1982722032076752220

 PubMed Web of Science ®Google Scholar

AbbasiNROrlowSJLichen planopilaris noted during etanercept therapy in a child with severe psoriasisPediatr Dermatol2009261118

 Web of Science ®Google Scholar

GarcovichSMancoSZampettiAAmerioPGarcovichAOnset of lichen planopilaris during treatment with etanerceptBr J Dermatol200815851161116318363764

 PubMed Web of Science ®Google Scholar

IsaacMMcNeelyMCDermatitis herpetiformis associated with lichen planopilarisJ Am Acad Dermatol1995336105010517490354

 PubMed Web of Science ®Google Scholar

JayasekeraPSWalshMLHurrellDParslewRACase report of lichen planopilaris occurring in a pediatric patient receiving a tumor necrosis factor α inhibitor and a review of the literaturePediatr Dermatol2016332e143e14626840781

 PubMed Web of Science ®Google Scholar

LaneTKKaminoHWaltersRFMeehanSPomeranzMKLichen planopilaris and psoriasisDermatol Online J200814104

 Google Scholar

RosinaPChieregatoCMagnaniniMBarbaALichen planopilaris and autoimmune thyroiditisJ Eur Acad Dermatol Venereol2002166648649

 Web of Science ®Google Scholar

BaibergenovaAWalshSUse of pioglitazone in patients with lichen planopilarisJ Cutan Med Surg20121629710022513061

 PubMed Web of Science ®Google Scholar

SpringPSpanouZde ViraghPALichen planopilaris treated by the peroxisome proliferator activated receptor-γ agonist pioglitazone: lack of lasting improvement or cure in the majority of patientsJ Am Acad Dermatol201369583083224124823

 PubMed Web of Science ®Google Scholar

MesinkovskaNATellezADawesDPiliangMBergfeldWThe use of oral pioglitazone in the treatment of lichen planopilarisJ Am Acad Dermatol201572235535625592345

 PubMed Web of Science ®Google Scholar

ChoBKSahDChwalekJEfficacy and safety of mycophenolate mofetil for lichen planopilarisJ Am Acad Dermatol201062339339720061053

 PubMed Web of Science ®Google Scholar

TursenUApiHKayaTIkizogluGTreatment of lichen planopilaris with mycophenolate mofetilDermatol Online J200410124

 PubMedGoogle Scholar

FerraraRJByrdRCLichen planopilaris: (follicular lichen planus)Cutis197312869870

 Google Scholar

Muñoz-PérezMACamachoFLichen planopilaris and scleroderma en coup de sabreJ Eur Acad Dermatol Venereol200216554254412428864

 PubMed Web of Science ®Google Scholar

MetinACalkaOUgrasSLichen planopilaris coexisting with erythema dyschromicum perstansBr J Dermatol2001145352252311531858

 PubMed Web of Science ®Google Scholar

AlmaaniNLiuLPerezARobsonAMellerioJEMcGrathJAEpidermolysis bullosa pruriginosa in association with lichen planopilarisClin Exp Dermatol2009348e825e82820055845

 PubMed Web of Science ®Google Scholar

GeorgeSJHsuSLichen planopilaris treated with thalidomideJ Am Acad Dermatol200145696596611712051

 PubMed Web of Science ®Google Scholar

JouaniqueCReygagnePBachelezHDubertretLThalidomide is ineffective in the treatment of lichen planopilarisJ Am Acad Dermatol2004513480481

 Web of Science ®Google Scholar