Table 1.
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 al8 | 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 Three withdrawn patients |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 al10 | Case series | 29 | IV | None | NA | 12 months | 4% (4 of 29) responders 72% (21 of 29) partial responders |
Lyakhovitsky et al1 | 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 al11 | Case series | 22 | IV | None | 6.5 mg/kg/day | 6–12 months | Nine patients showed improvement |
Assouly and Reigagne2 | Case series | 12 | IV | None | 400 mg/day | 6 months | No success |
Donati et al9 | 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 al12 | Case series | 9 | IV | None | NA | NA | Two patients showed improvement |
Mirmirani et al13 | Case series | 3 | IV | Intralesional and topical corticosteroids | NA | NA | No effect |
Mirmirani and Karnik14 | 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 al8 | 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 One withdrawn patient.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 al11 | 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 al1 | 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 al12 | Case series | 20 | IV | None | NA | NA | 14 responders |
Chieregato et al15 | 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 al16 | Case series | 2 | IV | Intralesional triamcinolone acetonide | Twice daily | NA | Decrease in symptoms and altering of skin lesion progression |
None | NA | NA | |||||
Abbasi and Orlow17 | Case report | 1 | V | Topical tacrolimus | NA | 2 years | Symptom relief but no clinical improvement |
Garcovich et al18 | Case report | 1 | V | Cyclosporine | NA | 7 months | Symptoms relief, but disease progression |
Isaac and McNeely19 | Case report | 1 | V | Intralesional corticosteroids, systemic corticosteroids, and griseofulvin | NA | NA | Symptom relief and disease stabilization |
Jayasekera et al20 | Case report | 1 | V | None | NA | NA | Improvement |
Lane et al21 | Case report | 1 | V | Cyclosporine | Thrice daily | NA | Symptom relief |
Rosina et al22 | 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 Walsh25 | Case series | 24 | IV | None | 15 mg/day | NA | Five patients with remission and 12 experiencing some improvement |
Spring et al27 | 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 al26 | Case series | 22 | IV | None | 15 mg/day | Median of 10.5 months | Marked improvement in 16 patients |
Mirmirani and Karnik14 | 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 al28 | 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 al11 | Case series | 10 | IV | None | 2–6 g/day | 3–6 months | Three patients showed improvement |
Assouly and Reigagne2 | Case series | 5 | IV | None | 2 g/day | 2–8 months | Improvement in two patients |
Tursen et al29 | 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 Karnik14 | 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 al11 | Case series | 15 | IV | None | 200 mg/day (doxycycline) | 3–6 months | Four patients showed improvement |
Lyakhovitsky et al1 | 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 al12 | Case report | 1 | IV | None | NA | NA | No improvement |
Ferrara and Byrd24 | Case report | 1 | V | None | NA | NA | Partial response |
Mirmirani and Karnik14 | 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 al1 | 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 al12 | Case series | 7 | IV | None | NA | NA | No responders |
Horn et al16 | 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 McNeely19 | Case report | 1 | V | Clobetasol propionate + systemic corticosteroids and griseofulvin | 10 mg/mL | NA | Symptom relief and disease stabilization |
Muñoz-Pérez and Camacho23 | Case report | 1 | V | None | NA | 8-month follow-up | Improvement, but small areas of permanent alopecia remained |
Ferrara and Byrd24 | 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 Reigagne2 | Case series | 13 | IV | None | 4–5 mg/kg/day | 4 months | Ten patients showed clinical improvement |
Mirmirani et al13 | Case series | 3 | IV | None | 3–5 mg/kg/day | 3–5 month | Improvement |
Mehregan et al12 | Case series | 2 | IV | None | NA | NA | No improvement |
Chieregato et al15 | Case series | 2 | IV | Topical steroids | 5 mg/kg/day for 15 days, then 3 mg/kg/day | 45 days | Improvement |
Garcovich et al18 | Case report | 1 | V | Topical betamethasone | 3 mg/kg/day | 7 months | Improvement |
Lane et al21 | 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 Reigagne2 | Case series | 6 | IV | None | 25 mg/day | NA | No success |
Lyakhovitsky et al1 | 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 al11 | 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 al12 | Case series | 11 | IV | None | 30–40 mg/day for at least 3 months with gradual taper | NA | Improvement in nine patients |
Lyakhovitsky et al1 | Case report | 1 | IV | None | NA | NA | No effect |
Isaac and McNeely19 | Case report | 1 | V | Intralesional corticosteroids, clobetasol propionate, and griseofulvin | NA | 4–6 weeks | Symptom relief and disease stabilization |
Ferrara and Byrd24 | Case report | 1 | V | None | NA | NA | Partial response |
Mirmirani and Karnik14 | 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 al12 | Case series | 10 | IV | None | NA | NA | Five patients showed improvement |
Isaac and McNeely19 | Case report | 1 | V | Intralesional corticosteroids, systemic corticosteroids, and clobetasol propionate | 1 g/day | NA | No improvement |
Metin et al30 | 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 al1 | 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 Orlow17 | Case report | 1 | V | None | NA | 2 years | Symptoms relief, but no clinical improvement |
Almaani et al31 | 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 al32 | Case series | 4 | IV | None | 100 mg/day for 1 month, then 200 mg/day | 6 months | No clinical improvement |
Assouly and Reigagne2 | Case series | 4 | IV | None | 100 mg/day | 6 months | No success |
George and Hsu31 | 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 al33 | 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