Key Points
Question
What is the overall prevalence of lichen planopilaris (LPP), and what treatments are patients with LPP receiving?
Findings
In this cross-sectional study, the standardized overall prevalence of LPP was 13.4 per 100 000. Most of the 991 patients with LPP received topical/intralesional corticosteroids or systemic medications, and a substantial number were treated for more than 1 year, required combination treatment, and underwent treatment switching.
Meaning
LPP is a skin disease that frequently requires long-term treatment, combination treatment, and treatment switching; these findings highlight the lack of a reliable, approved treatment for patients with LPP.
Abstract
Importance
Lichen planopilaris (LPP) is a form of scarring alopecia associated with progressive, permanent hair loss. Symptoms range from burning pain to itching, also carrying substantial psychological morbidity. Yet, disease characteristics, pathophysiology, and effective treatment data are limited, making treatment a challenge.
Objective
To describe the prevalence and dermatologist-prescribed treatment patterns of LPP among US adults.
Design, Setting, and Participants
This cross-sectional study used the Explorys database. The prevalence analysis used a 15% random sample and identified US adults with LPP diagnoses between 2017 and 2019. The LPP treatment analysis included all patients with LPP diagnoses between 2016 and 2020 and a dermatologist encounter in the first year after diagnosis. Data were analyzed from January 2023 to April 2023.
Main Outcomes and Measures
The main outcomes of the prevalence analysis were the crude and standardized prevalence estimates of US adults with LPP across age, sex, and racial groups. The main outcomes of the treatment analysis were the frequency of LPP treatments within 1 year of diagnosis, and the number of patients who continued treatment beyond 1 year, switched treatments, and combined treatments.
Results
Among 1 466 832 eligible patients analyzed for prevalence, 241 patients had an LPP diagnosis (222 [92.1%] female; median [IQR] age, 64 [54-73] years). Standardized overall prevalence was 13.4 per 100 000 (95% CI, 11.7-15.1). In the treatment analysis, 991 patients had an LPP diagnosis (907 [91.5%] female; median (IQR) age, 60 [47-69] years). Most received at least 1 type of medication (635 [64.1%]), most frequently intralesional corticosteroids (370 [37.3%]) and topical corticosteroids (342 [34.5%]), followed by doxycycline (104 [10.5%]) and hydroxychloroquine (72 [7.3%]). Treatment continued beyond 1 year in 71 of 200 patients (35.5%) prescribed intralesional corticosteroids and 7 of 29 patients (24.1%) prescribed hydroxychloroquine. Treatment switching at 1 year occurred in 32 of 254 patients (12.6%) first prescribed an intralesional corticosteroid and in 44 of 194 (22.7%) first prescribed a topical corticosteroid. Combinations of 2 or 3 treatment types were given to 137 (13.8%) and 74 (7.5%) patients, respectively.
Conclusions and Relevance
This cross-sectional study reported prevalence and treatment patterns for US adults with LPP in a representative sample. Most patients with LPP received treatment, and many received multiple treatment types and switched treatments, suggesting further research into medication selection offers clinical benefit.
This cross-sectional study evaluates the prevalence of lichen planopilaris and analyzes treatment patterns among a representative US-based database.
Introduction
Lichen planopilaris (LPP) is a morphological variant of lichen planus characterized by inflammation-driven hair follicle destruction and subsequent replacement with fibrous tissue.1 Patients experience scarring and hair loss associated with itching, tenderness, and burning, significantly decreasing quality of life.2 Robust epidemiologic and pathophysiologic data are limited with no evidence-based approved treatments.3 Our objective was to estimate the prevalence of LPP in the US health care–seeking population and assess dermatologist-prescribed treatment patterns.
Methods
This cross-sectional study used the Explorys database, which has electronic medical record data from more than 40 health care delivery networks and 53 million patients across the US.4 Institutional review board approval and informed consent were waived in this study because the data were deidentified. Data were analyzed from January 2023 to April 2023.
We used a 15% random sample of all patients in the prevalence analysis. Patients with LPP were identified by at least 1 recorded code (L66.1) from the International Statistical Classification of Diseases and Related Health Problems, Tenth Revision (ICD-10) on or before December 31, 2019. In a separate validation study, we observed a positive predictive value of 87.8% for the methods used to diagnose patients with LPP.5
A 100% sample of patients diagnosed with LPP from the Explorys database made up the cohort. Patients with a new diagnosis of LPP between April 1, 2016, and January 1, 2020, and at least 1 LPP-associated dermatology encounter during the first year following diagnosis were included. In a validation study from Northwell Health, at least 1 LPP code by a dermatologist yielded a positive predictive value of 93.0%.5 Combination treatments, treatment switching, and treatment continuation are defined in the eMethods in Supplement 1.
R, version 3.6.3 (R Project for Statistical Computing), was used for this analysis. Statistical significance, determined with 2-tailed, unpaired testing, was evaluated at the α < .05 level.
Results
Prevalence
The prevalence analysis comprised 241 patients with LPP among 1 466 832 eligible patients in the database (222 [92.1%] female; median [IQR] age, 64 [54-73] years). The crude prevalence of LPP was 16.4 per 100 000 (95% CI, 14.5-18.6) (Table 1). Age-standardized and sex-standardized overall prevalence was 13.4 per 100 000 (95% CI, 11.7-15.1). Age-standardized prevalence was greater in female than male patients (22.7 per 100 000 [95% CI, 19.7-25.7] vs 2.9 per 100 000 [95% CI 1.6-4.3]). Standardized prevalence of LPP also varied within age groups; the highest prevalence was observed in those aged between 70 and 79 years (25.8 per 100 000).
Table 1. Overall and Demographic Subgroup Prevalence of LPP.
| Subgroup | Patients with LPP, No. | Overall patients, No. | Crude prevalence per 100 000 (95% CI) | Standardized prevalence per 100 000 (95% CI)a |
|---|---|---|---|---|
| Total | 241 | 1 466 832 | 16.4 (14.5-18.6) | 13.4 (11.7-15.1) |
| Sex | ||||
| Female | 222 | 870 266 | 25.5 (22.4-29.1) | 22.7 (19.7-25.7) |
| Male | 19 | 596 566 | 3.2 (2.0-5.0) | 2.9 (1.6-4.3) |
| Age, y | ||||
| 18-29 | 5 | 232 401 | 2.2 (0.9-5.0) | 2.2 (0.2-4.1) |
| 30-39 | 13 | 208 127 | 6.2 (3.7-10.7) | 5.3 (2.4-8.3) |
| 40-49 | 34 | 210 674 | 16.1 (11.5-22.6) | 14.3 (9.5-19.1) |
| 50-59 | 37 | 255 988 | 14.5 (10.5-19.9) | 13.1 (8.9-17.4) |
| 60-69 | 67 | 260 828 | 25.7 (20.2-32.6) | 23.6 (18.0-29.3) |
| ≥70 | 85 | 298 814 | 28.4 (23.0-35.2) | 25.8 (20.3-31.3) |
| Raceb | ||||
| Asian | 3 | 19 026 | 15.8 (5.4-46.4) | NA |
| Black | 41 | 184 572 | 22.2 (16.4-30.1) | 18.5 (12.8-24.2) |
| White | 163 | 1 056 283 | 15.4 (13.2-18.0) | 11.9 (10.0-13.8) |
| Otherc | 12 | 93 698 | 12.8 (7.3-22.4) | NA |
Abbreviations: LPP, lichen planopilaris; NA, not applicable.
Prevalence data were directly standardized using population estimates from the 2019 American Community Survey from the US Census Bureau. Sex-specific estimates were standardized by age. Age-specific estimates were standardized by sex. Race-specific estimates were standardized by age and sex.
Race was self-reported in the Explorys database. A total of 113 253 patients missing race data were excluded from the race-specific analysis. Patients missing race were included in the estimates of LPP prevalence for the overall group, sex subgroup, and age subgroup.
Asian/Pacific Islander, Native American or Alaska Native, Native Hawaiian, multiracial, or not specified.
Treatment Patterns
Overall, 991 patients had an LPP-associated dermatology encounter in the database (907 [91.5%] female; median (IQR) age, 60 [47-69] years) (eTable 2 in Supplement 1). Of these, 635 (64.1%) received at least 1 type of medication, most frequently intralesional corticosteroids (ILC) (370 [37.3%]) and topical corticosteroids (TCS) (342 [34.5%]), followed by oral doxycycline (104 [10.5%]) and hydroxychloroquine (72 [7.3%]) (Table 2). Other treatments were infrequently prescribed, such as 5-α reductase inhibitors (23 [2.3%]), systemic immunosuppressants (11 [1.1%]), and pioglitazone (3 [0.3%]). No patients met the criteria for JAK inhibitor prescriptions during the study.
Table 2. Overall Treatment Data and Continuation of Therapy for Patients With LPP.
| Treatmenta | Patients receiving treatment within 1 y of LPP diagnosis, No. (%) (n = 991) | Patients continuing treatment 1 y after initial prescription, No./total No. (%)b |
|---|---|---|
| Intralesional corticosteroid | 370 (37.3) | 71/200 (35.5) |
| Topical corticosteroid | 342 (34.5) | 35/155 (22.6) |
| Doxycycline | 104 (10.5) | 8/48 (16.7) |
| Hydroxychloroquine | 72 (7.3) | 7/29 (24.1) |
| Topical calcineurin inhibitors | 51 (5.1) | 6/26 (23.1) |
| Systemic corticosteroid | 40 (4.0) | 2/18 (11.1) |
| 5-α Reductase inhibitors | 23 (2.3) | 0/7 |
| Systemic immunosuppressants | 11 (1.1) | 0/5 |
| Pioglitazone | 3 (0.3) | 0/1 |
| JAK inhibitor (tofacitinib) | 0 | NA |
Abbreviations: JAK, Janus kinase; LPP, lichen planopilaris; NA, not applicable.
Any prescription associated with a dermatology encounter for LPP within 7 days of an LPP diagnosis for the first year following the initial LPP diagnosis.
Denominator for percentages is the number of patients with at least 1 year of follow-up after the initial prescription.
Patients receiving treatment most often received 1 treatment category within 1 year of diagnosis (395 [39.9%]). Many patients received 2 or 3 treatment types (137 [13.8] and 74 [7.5%], respectively). The most common initial treatments were ILC (301 [47.4%]) and TCS (299 [47.1%]). Treatment continuation was most often observed in patients taking an ILC (71 of 200 [35.5%] with 1-year follow-up) or hydroxychloroquine (7 of 29 [24.1%] with 1-year follow-up). In the first year of treatment, 201 patients (20.3%) were prescribed combination therapy (eTable 3 in Supplement 1). The most frequent combination regimens were ILC with TCS (90 [44.8%]) and TCS with oral doxycycline (64 [31.8%]). Combinations of ILC or TCS with hydroxychloroquine were less common (34 [16.9%] and 31 [15.4%], respectively; Table 3). The highest proportion of patients switching to a different or combination therapy within 12 months initially received hydroxychloroquine (7 of 10), systemic steroids (8 of 18), systemic immunosuppressants (1 of 3), and oral doxycycline (7 of 20). Treatment switching at 1 year occurred in 32 of 254 patients (12.6%) with an initial ILC prescription, and in 44 of 194 patients (22.7%) with an initial TCS prescription (Table 3; eTable 4 in Supplement 1).
Table 3. Initial Treatments Prescribed After Lichen Planopilaris Diagnosis and Treatment Switchinga.
| First treatment | Patients with initial treatment prescription, No. (%) (n = 635)b | Switching from initial treatment, No./total No. (%)c | |
|---|---|---|---|
| 6 mo | 12 mo | ||
| Intralesional corticosteroid | 301 (47.4) | 23/254 (9.1) | 32/254 (12.6) |
| Topical corticosteroid | 299 (47.1) | 35/194 (18.0) | 44/194 (22.7) |
| Doxycycline | 71 (11.2) | 5/20 (25.0) | 7/20 (35.0) |
| Hydroxychloroquine | 36 (5.7) | 4/10 (40.0) | 7/10 (70.0) |
| Topical calcineurin inhibitors | 30 (4.7) | 2/11 (18.2) | 2/11 (18.2) |
| Systemic corticosteroid | 22 (3.5) | 6/18 (33.3) | 8/18 (44.4) |
| 5-α Reductase inhibitors | 14 (2.2) | 0/3 | 1/3 (33.3) |
| Systemic immunosuppressants | 7 (1.1) | 1/3 (33.3) | 1/3 (33.3) |
| Pioglitazone | 0 | NA | NA |
| JAK inhibitor (tofacitinib) | 0 | NA | NA |
| Initial prescription for ≥2 treatments herein | 122 (19.2) | NA | NA |
Abbreviations: JAK, Janus kinase; NA, not applicable.
Treatment switching was defined as a prescription for a different treatment type than the initial treatment at least 30 days after the initial treatment. This definition includes a switch to a new treatment or the addition of a different treatment for combination therapy.
Denominator includes patients prescribed at least 1 of the treatments listed during the 1 year after the first LPP diagnosis.
Patients with more than 1 initial treatment (eg, combination) were excluded from this analysis.
Discussion
This cross-sectional study used a representative US database to characterize LPP. We calculated the overall standardized prevalence of LPP among US adults, 13.4 per 100 000 people. Consistent with previous research, LPP was prevalent in women and patients older than 60 years.6,7,8,9,10 Population-based studies that have explored the overall and subgroup prevalence of LPP are sparse, often limited by a lack of standardization in prevalence calculations.6,8
Randomized clinical trials for treating patients with LPP are absent, and evidence for treatments is primarily based on case reports and case series, often showing suboptimal or no therapeutic response.3,11,12 In this study, we have described LPP treatment patterns in routine dermatology practice in the US.
Consistent with published data, most patients received at least 1 treatment in this study.10 The most common initial and overall treatments for LPP were ILC and TCS, followed by oral doxycycline, consistent with previous studies.11,12,13 The chronic, progressive nature of LPP may explain the need for treatment continuation. Both ILC and TCS treatments were commonly used as long-term therapy, but patients frequently switched between them, potentially representing treatment escalation/de-escalation.
In patients who do not respond to first-line treatment, other systemic medications may be considered.13 Hydroxychloroquine as continued therapy and systemic steroids as short-term noncontinued treatment reflect clinical practice. The higher likelihood of treatment switching and lower frequency of treatment continuation in patients initially prescribed immunosuppressants (eg, methotrexate, mycophenolate, and cyclosporine) may have represented patients with severe, refractory disease. These data suggest that improved stratification of patients requiring systemic treatment and further development of safe, effective long-term therapies is needed.
Limitations
The limitations of this study included missing data and potential miscoding. The study period was limited by LPP lacking an ICD-10 code until late 2015 and health care disruptions due to the COVID-19 pandemic. Prevalence in the health care–seeking population may be overestimations in the general population. Differentiating subtypes sharing the same ICD-10 code, including frontal fibrosing alopecia, and stratifying treatments by disease severity or extent was not feasible in a claims database study. The 7-day limit for prescriptions may have missed prescriptions with a delay from a biopsy result, for example, and patients who were treated using drug samples or assistance programs.
Conclusions
This cross-sectional study sought to characterize prevalence and treatment patterns in a large, representative US database. The overall standardized prevalence of LPP was 13.4 per 100 000 US adults. LPP is prevalent among female and older patients. Most patients with LPP received at least 1 dermatologist-prescribed treatment; approximately 20% of patients received combination therapy at the initial visit. Monotherapies with ILC and TCS were the most common initial and overall treatments, followed by doxycycline and hydroxychloroquine. Many patients continued treatment past 1 year, primarily patients receiving ILC or hydroxychloroquine. Patients initially treated with hydroxychloroquine or systemic therapy were more likely to switch to a different medication or combination therapy within 1 year. Future research is needed to optimize treatment regimens and develop new, targeted therapies for patients with LPP to limit its morbidity and permanent sequelae.
eMethods
eReferences
eTable 1. Lichen planopilaris treatment categories and corresponding treatment names
eTable 2. Lichen planopilaris treatments: patient demographics
eTable 3. Treatments prescribed by a dermatologist within 1 year of LPP diagnosis according to age
eTable 4. Specific treatment switches at 12 months for patients initially prescribed topical corticosteroids and intralesional corticosteroids
Data Sharing Statement
References
- 1.Harries MJ, Paus R. The pathogenesis of primary cicatricial alopecias. Am J Pathol. 2010;177(5):2152-2162. doi: 10.2353/ajpath.2010.100454 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Nasimi M, Ahangari N, Lajevardi V, Mahmoudi H, Ghodsi SZ, Etesami I. Quality of life and mental health status in patients with lichen planopilaris based on Dermatology Life Quality Index and General Health Questionnaire-28 questionnaires. Int J Womens Dermatol. 2020;6(5):399-403. doi: 10.1016/j.ijwd.2020.09.005 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Rácz E, Gho C, Moorman PW, Noordhoek Hegt V, Neumann HA. Treatment of frontal fibrosing alopecia and lichen planopilaris: a systematic review. J Eur Acad Dermatol Venereol. 2013;27(12):1461-1470. doi: 10.1111/jdv.12139 [DOI] [PubMed] [Google Scholar]
- 4.Health IW. The IBM Explorys platform: liberate your healthcare data. Accessed March 31, 2023. https://ftpmirror.your.org/pub/misc/ftp.software.ibm.com/common/ssi/ecm/hp/en/hps03052usen/watson-health-healthcare-providers-hp-solution-brief-hps03052usen-20180316.pdf
- 5.Pelet Del Toro N, Strunk A, Garg A, Han G. Validity of lichen planus and lichen planopilaris case identification using diagnostic codes from a clinical database. Dermatology. 2023;239(6):906-911. doi: 10.1159/000533247 [DOI] [PubMed] [Google Scholar]
- 6.Trager MH, Lavian J, Lee EY, et al. Prevalence estimates for lichen planopilaris and frontal fibrosing alopecia in a New York City health care system. J Am Acad Dermatol. 2021;84(4):1166-1169. doi: 10.1016/j.jaad.2020.10.081 [DOI] [PubMed] [Google Scholar]
- 7.Schruf E, Biermann MH, Jacob J, et al. Lichen planus in Germany—epidemiology, treatment, and comorbidity: a retrospective claims data analysis. J Dtsch Dermatol Ges. 2022;20(8):1101-1110. doi: 10.1111/ddg.14808 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Joshi TP, Zhu H, Naqvi Z, Holla S, Duruewuru A, Ren V. Prevalence of lichen planopilaris in the United States: a cross-sectional study of the All of Us research program. JAAD Int. 2022;8:69-70. doi: 10.1016/j.jdin.2022.05.003 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Meinhard J, Stroux A, Lünnemann L, Vogt A, Blume-Peytavi U. Lichen planopilaris: epidemiology and prevalence of subtypes—a retrospective analysis in 104 patients. J Dtsch Dermatol Ges. 2014;12(3):229-236. doi: 10.1111/ddg.12264 [DOI] [PubMed] [Google Scholar]
- 10.Mehregan DA, Van Hale HM, Muller SA. Lichen planopilaris: clinical and pathologic study of forty-five patients. J Am Acad Dermatol. 1992;27(6 Pt 1):935-942. doi: 10.1016/0190-9622(92)70290-V [DOI] [PubMed] [Google Scholar]
- 11.Spencer LA, Hawryluk EB, English JC III. Lichen planopilaris: retrospective study and stepwise therapeutic approach. Arch Dermatol. 2009;145(3):333-334. doi: 10.1001/archdermatol.2008.590 [DOI] [PubMed] [Google Scholar]
- 12.Cevasco NC, Bergfeld WF, Remzi BK, de Knott HR. A case-series of 29 patients with lichen planopilaris: the Cleveland Clinic Foundation experience on evaluation, diagnosis, and treatment. J Am Acad Dermatol. 2007;57(1):47-53. doi: 10.1016/j.jaad.2007.01.011 [DOI] [PubMed] [Google Scholar]
- 13.Babahosseini H, Tavakolpour S, Mahmoudi H, et al. Lichen planopilaris: retrospective study on the characteristics and treatment of 291 patients. J Dermatolog Treat. 2019;30(6):598-604. doi: 10.1080/09546634.2018.1542480 [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
eMethods
eReferences
eTable 1. Lichen planopilaris treatment categories and corresponding treatment names
eTable 2. Lichen planopilaris treatments: patient demographics
eTable 3. Treatments prescribed by a dermatologist within 1 year of LPP diagnosis according to age
eTable 4. Specific treatment switches at 12 months for patients initially prescribed topical corticosteroids and intralesional corticosteroids
Data Sharing Statement
