Multisite lichen planopilaris, oral lichen planus, and vulvar lichen sclerosus treated using Janus kinase inhibitors

What is known about this subject in regard to women and their families?

• Lichen planopilaris, oral lichen planus, and vulvar lichen sclerosus are chronic inflammatory conditions that can occur together and may be difficult to control with standard treatments.

• These conditions can cause substantial symptoms, including scalp hair loss, pain, burning, itching, and oral or vulvar discomfort, which may negatively affect quality of life.

What is new from this article as messages for women and their families?

• In this small case series, oral Janus kinase (JAK) inhibitor therapy improved symptoms and disease activity across multiple affected sites in patients with overlapping lichenoid conditions.

• Our findings suggest that systemic JAK inhibition may be a treatment option for patients with severe or refractory multisite lichenoid disease, although future larger studies are needed to confirm long-term outcomes.

Concurrent lichen planopilaris (LPP), oral lichen planus, vulvar lichen sclerosus, and cutaneous lichen planus present a rare, complex mucocutaneous systemic disease. Their coexistence reflects their shared immune-mediated pathways and is frequently refractory to standard therapies, including corticosteroids and traditional immunomodulators, underscoring the need for targeted systemic agents.^1,2 Dysregulated Janus kinase (JAK)-STAT signaling has been implicated in the pathogenesis of each condition, providing a mechanistic rationale for JAK inhibitor therapy.^3,4 We present 4 cases demonstrating the clinical utility of JAK inhibitors for multisite lichenoid disease.

We conducted a retrospective case series of 4 women aged 43–68 with biopsy-confirmed LPP and concomitant oral lichen planus, vulvar lichen sclerosus, or cutaneous lichen planus who were treated with a systemic JAK inhibitor (baricitinib or tofacitinib) at Lahey Hospital and Medical Center. All patients had failed multiple therapies, including topical, systemic, and intralesional corticosteroids; calcineurin inhibitors; hydroxychloroquine; systemic immunomodulators; and topical JAK inhibitors. Clinical response was evaluated at 6–12 week intervals based on symptom burden, trichoscopy, and standardized scalp photography. All patients remained on therapy at last follow-up. Key findings are summarized in Table 1.

Table 1.

Summary of key findings of patients with multisite lichenoid disease treated with JAK inhibitors

Feature	Patient 1	Patient 2	Patient 3	Patient 4
Age	63	68	43	61
Diagnoses	LPP (scalp) Vulvar lichen sclerosus Oral lichen planus	LPP (scalp) Vulvar lichen sclerosus Oral lichen sclerosus	LPP (scalp) Vulvar lichen planus Oral lichen planus Cutaneous lichen planus (wrists)	LPP (scalp) Vulvar lichen sclerosus Oral lichen planus Cutaneous lichen planus (wrists)
Prior therapies	Topical steroids Tacrolimus Mycophenolate Prednisone Fluocinonide solution Topical minoxidil	HCQ Doxycycline Clobetasol foam ILK injections (last Jan 2024)	HCQ (d/c by ophtho) Doxycycline ILK Clobetasol nbUVB Tacrolimus solution Roflumilast foam	Oral minoxidil 1.25 mg QD
Concurrent therapies	Oral minoxidil 2.5 mg QOD (beginning Nov 2024) Fluocinonide BIW (beginning Oct 2023)	Oral minoxidil 2.5 mg QD (beginning Sept 2022) Ketoconazole shampoo TIW (Aug 2024) Topical nystatin/clobetasol ointment PRN (beginning Nov 2022) for vulvar lichen sclerosus	–	HCQ 200 mg QD (beginning April 2021) Oral minoxidil 3.75 mg QD (beginning Oct 2025) Clobetasol solution BIW-TIW (beginning Sept 2024) Topical ruxolitinib BIW-TIW (beginning Sept 2024) Topical minoxidil 5% foam BIW (beginning Feb 2022)
JAK inhibitor	Baricitinib 4 mg QD	Baricitinib 4 mg QD	Tofacitinib 5 mg BID	Tofacitinib 10 mg BID
Duration of treatment with JAK inhibitor	20 months (June 2024–Feb 2026)	45 months (Sept 2022–June 2026)	42 months (Nov 2022–May 2026)	15 months (Jan 2025–April 2026)a
Scalp response	Stable at 6 months, improvement in 12 months with ↑ hair density and ↓ pain/pruritus	Erythema/scale 2→0 at 6 weeks; follicular rescue and ↓ pain/pruritus at 11 weeks	0% scalp affected; ↑ hair density and ↓ pain/pruritus at 2 years	Erythema/scale 0; approximately 2% scalp at 9 months; <5% with ↑ density at 15 months
Vulvar response	Significant improvement at approximately 12 months	Significant improvement at 11 weeks	Significant improvement at 2 years	Improved by approximately 9 months; well controlled at 15 months
Oral response	Significant improvement at approximately 12 months	Improved at 11 weeks	Significant improvement at 2 years	Improved by approximately 9 months; well controlled at 15 months
Cutaneous lichen planus (wrist) response	–	–	Resolved at approximately 1.5 years	Resolved at approximately 1.5 years
Adverse events	Increased acne	Tinea capitis × 2 (Jan 2024, resolved with econazole; Aug 2024, resolved with terbinafine PO)	Biliary colic	None reported
Notes	–	–	–	↓ Scalp pruritus and redness at 9 months; aReduction to 10 mg QD (Oct 2025–Jan 2026) caused symptom recurrence in all affected sites; restored to 10 mg BID with resolution; taper to 5 mg BID planned

BID, twice-daily dosing; BIW, twice weekly; d/c, discontinued; HCQ, hydroxychloroquine; ILK, intralesional kenalog; JAKi, Janus kinase inhibitor; LPP, lichen planopilaris; nbUVB, narrowband ultraviolet B; ophtho, ophthalmology; PRN, as needed; QD, once daily dosing; TIW, thrice weekly.

Timepoints reflect the visit at which clinically notable improvement was first documented; all patients were followed at routine 4–8 month intervals.

^aPatient 4’s tofacitinib dose was reduced to 10 mg once daily from October 2025 to January 2026, which resulted in symptom recurrence at all affected sites. The dose was subsequently returned to 10 mg twice daily, with subsequent resolution of symptoms.

All 4 patients demonstrated significant clinical improvement following the initiation of oral JAK inhibitor therapy. Scalp inflammation, mucosal disease, and cutaneous involvement (if applicable) resolved or markedly improved in each case. Hair loss was also reduced, with 3 patients experiencing increased hair density (Fig. 1). Patient-reported symptoms, including pain, burning, and pruritus, improved in all 4 cases. Treatment was well tolerated. Adverse effects included acne, tinea corporis, and biliary colic, all of which were limited and manageable. No serious laboratory abnormalities were observed. Notably, Patient 4 experienced symptom recurrence at all affected sites when reducing tofacitinib from 10 mg twice daily to 10 mg once daily, which resolved upon returning to the twice-daily dose.

Fig. 1.

Global scalp photography of Patient 1, a 63-year-old woman with multisite lichenoid disease. (Left) Baseline scalp photography showing large patches of scarring alopecia before starting oral JAK inhibitor therapy (June 2024). (Right) Scalp photography at most recent follow-up after 45 months of treatment on oral baricitinib 4 mg daily, showing increased hair density (May 2025). JAK, Janus kinase.

LPP, oral lichen planus, vulvar lichen sclerosus, and cutaneous lichen planus share overlapping immunopathology characterized by dysregulated T-cell activation and cytokine signaling through the JAK-STAT pathway.^2–4 This mechanistic convergence supports systemic JAK inhibition as a unifying therapeutic strategy across disease sites. Prior reports have documented the efficacy of JAK inhibitors in each of these conditions separately.^3–5 The simultaneous multisite responses observed across all 4 patients in this study suggest a shared inflammatory driver targeted by JAK inhibitors, rather than independent, site-specific diseases. This distinction has important clinical implications, as patients with overlapping lichenoid diseases may be suboptimally managed by separate site-directed therapies and could benefit from earlier initiation of systemic treatment. The observed symptom worsening in Patient 4 after decreasing her JAK inhibitor dosing frequency further supports the role of optimized dosing in sustained disease. The concurrent improvement across each disease further supports this concept and adds to the existing literature on JAK inhibitors in overlapping lichenoid diseases.

Oral JAK inhibitors offer an effective, well-tolerated option for patients with refractory multisite lichenoid disease. Limitations of this series include its retrospective design, small sample size, lack of systematic measures, and potential confounding by adjunctive therapies. Larger, prospective studies are needed to confirm the long-term efficacy and safety of this approach.

Conflicts of interest

The authors made the following disclosures: M.M.S. has been an investigator for Q32Bio, Inmagene, Abbvie, Priovant, Leo Pharma, and Aldena. She has been on SAB or consulted for Lilly, Pfizer, Abbvie, Inmagene, Veradermics, and Priovant.

Funding

None.

Study approval

This study was approved by the Institutional Review Board of Lahey Hospital and Medical Center. The requirement for informed consent was waived due to the retrospective nature of the study.

Author contributions

MP: Participated in conceptualization, methodology, validation, analysis, investigation, data curation, and writing – original draft, review, and editing. RZE: Participated in validation, methodology, and writing – review & editing. CK: Participated in methodology and writing – review & editing. MMS: Participated in conceptualization, validation, writing – review & editing, and supervision.