Case Report Demonstrates Treatment of Refractory Follicular Lichen Planus With Ruxolitinib

In the case of a patient with recalcitrant follicular lichen planus, also known as lichen planopilaris, researchers recently reported successful treatment and lesion clearance following 1 month of treatment with topical Janus kinase (JAK) inhibitor ruxolitinib (Opzelura; Incyte).

The case report, published in Journal of the European Academy of Dermatology and Venereology, presents clinicians with a potential off-label use of the topical therapy in a condition that currently has no approved treatment options.¹

Image Credit: © DermNet

Background and Methods

Lichen planopilaris is recognized in several forms, including follicular lichen planus, frontal fibrosing alopecia, and Graham Little syndrome. The rare inflammatory condition is associated with scarring and permanent hair loss. While its exact cause is unknown, but it is believed to involve an autoimmune response against hair follicles.²

Currently-utilized options include corticosteroids (both topical and systemic), immunosuppressive or immunomodulatory drugs, and antimalarial drugs. A 2019 retrospective study published in the Journal of Dermatological Treatment assessed numerous reported treatments and cases of follicular lichen planus. Patients treated with cyclosporine and methotrexate yielded the highest response rates, while mycophenolate mofetil also demonstrated efficacy with enhanced safety.³

The patient, a 62-year-old male, sought medical attention for inflammatory hair loss and scalp itching localized to the occipital region. His symptoms had persisted for over a year and a half without improvement despite the application of topical corticosteroids. The patient had also presented with asymmetric patches of scarring alopecia accompanied by perifollicular erythema and scaling.

Researchers conducted a scalp biopsy and histopathological analysis to aid in the diagnosis, finding lichenoid inflammatory infiltrate around the upper portion of hair follicles, vacuolar degeneration of keratinocytes, and necroptotic changes. They also observed lesional expression of Myxovirus Resistance A and recorded a Lichen Planus Activity and Damage Index (LiPADI) score of 5, indicative of severe disease.

Findings

Researchers initiated treatment with topical mometasone cream for 4 weeks. Following this, they introduced oral prednisolone at a dosage of 40 mg per day with a tapering schedule over 2 weeks. Despite these treatments, the disease remained active with continued follicular hyperkeratosis and inflammation. Researchers evaluated skin lesions with a reduced LiPADI score of 4.

Researchers then initiated therapy with topical ruxolitinib applied twice daily, citing its targeted mechanism of action against the inflammatory pathways involved in lichen planopilaris.

After 4 weeks of treatment with ruxolitinib, researchers observed significant clinical improvement. The patient's scalp lesions significantly reduced in both inflammation and follicular damage, and the LiPADI activity score decreased from 4 to 1.

Following the 4-week treatment period, researchers limited application frequency to every 1 to 2 days, with favorable, well-controlled results observed through 20 weeks.

Conclusions

"Topical ruxolitinib has been shown to be effective in a small case series of 12 patients with cutaneous LP as well as in one patient with recalcitrant LP pigmentosus," according to Fetter et al. "In addition, oral JAK inhibitors, including tofacitinib, baricitinib, ruxolitinib and upadacitinib, have been shown to reduce LP disease activity in recalcitrant LP."

Authors of the case report noted that it is worth closely following the development of the various JAK inhibitors in clinical development, as they may prove promising in other disease states outside of their approved indications.

References

1. Fetter T, Wilsmann-Theis D, de Vos-Hillebrand L, Novak N, Wenzel J. Successful treatment of recalcitrant follicular lichen planus (lichen planopilaris) with the topical JAK 1/2 inhibitor ruxolitinib. J Eur Acad Dermatol Venereol. August 14, 2024. https://doi.org/10.1002/jvc2.515
2. DermNet NZ. Lichen planopilaris. Accessed August 22, 2024. https://dermnetnz.org/topics/lichen-planopilaris
3. Babahosseini H, Tavakolpour S, Mahmoudi H, Balighi K, Teimourpour A, Ghodsi SZ, et al. Lichen planopilaris: retrospective study on the characteristics and treatment of 291 patients. J Dermatol Treatment. 2019; 30(6): 598–604. https://doi.org/10.1080/09546634.2018.15424