Recalcitrant vulvar lichen planus cleared on abrocitinib: A case report

Abstract

Lichen planus is a chronic inflammatory disorder, with vulvar variants often resistant to multiple treatments. The primary therapeutic goals for vulvar lichen planus are to prevent scarring and the development of squamous cell carcinoma. Management also involves treating associated symptoms such as pruritus and pain. This report describes a 50-year-old female with treatment-resistant hypertrophic and erosive vulvar lichen planus. Despite extensive trials of various therapies, the patient experienced symptoms of pruritus, pain, and repeated flares, significantly affecting her quality of life. Abrocitinib, a highly selective Janus kinase 1 inhibitor, was added to her treatment regimen, leading to a 95% resolution of lesions and substantial improvement in quality of life. This case highlights the potential of abrocitinib as an effective, novel off-label treatment for recalcitrant vulvar lichen planus.

Introduction

Lichen planus is a chronic inflammatory disease with its pathophysiology not fully understood. The disease can affect the nails, skin, hair, and mucosa, with most of the cutaneous cases improving in 1–2 years.^1,2 On the contrary, the follicular and mucosal subtypes can persist much longer. ² Genital lichen planus can present as classic, erosive, hypertrophic, or lichen planopilaris morphology. Given its recalcitrant nature, genital variants can severely impact a patient’s quality of life, leading to significant physical and psychological symptoms.^3,4 The primary therapeutic goals for vulvar lichen planus are to prevent the development of squamous cell carcinoma and vulvar and vaginal scarring, which can lead to vaginal stenosis, synechiae, and introitus narrowing. Management also involves treating associated symptoms of pruritus and pain. The erosive and hypertrophic disease forms can be refractory to numerous treatments, including topical corticosteroids, systemic anti-inflammatory medications, and immunosuppressants. ⁴ Due to a lack of robust clinical trials, ⁴ genital lichen planus treatment relies heavily on case reports. Despite the successful treatment of oral lichen planus and lichen planopilaris with Janus kinase (JAK) inhibitors in case reports, published literature on its use in vulvar lichen planus is lacking.^5,6 Moreover, there is no published literature on the use of the selective JAK1 inhibitor, abrocitinib, for this condition. This report details a case of treatment-resistant hypertrophic and erosive vulvar lichen planus, which showed significant improvement and almost complete resolution with the initiation of abrocitinib.

Case report

A 50-year-old female patient initially presented to the vulvar dermatology clinic with a 20-year history of extremely pruritic plaques accompanied by erosions and fissures of the vulvar vestibule. Biopsy confirmed a diagnosis of lichen planus. Past medical history at that time was significant only for type 1 neurofibromatosis. Previous initial treatment had included topical clobetasol propionate 0.05% ointment up to twice daily, which helped clear the posterior vestibule but was no longer effective for the anterior portion (Figure 1). She was then trialed on various treatments, including intralesional steroid injections 10 mg/mL, a prednisone taper over 40 days, methotrexate 25 mg oral weekly, and alitretinoin 10 mg oral daily. Given a rise in triglycerides on blood work and poor symptom control, methotrexate and alitretinoin were discontinued after 8 and 3 months, respectively. She was then started on cyclosporine at 150 mg/day, divided into two doses, and later titrated to 200 mg/day, alongside mycophenolate mofetil 1.5 g twice daily. Over the next several months, her symptoms continued to persist, and she was tapered off cyclosporine after 6 months of therapy. She continued mycophenolate mofetil, while acitretin 10 mg oral daily and apremilast 30 mg oral twice daily were initiated with about 30 to 40% initial improvement after 4 months. Despite adherence to her treatment regimen, she experienced repeated flares and persistent pruritus, severely impacting her daily functioning and sleep. Given her various treatment failures, mycophenolate mofetil was stopped after 15 months. A trial of abrocitinib 200 mg oral daily was added to her treatment regimen, which also included apremilast, acitretin, clobetasol propionate 0.05% ointment for flares, and tacrolimus 0.1% ointment daily for maintenance. After 8 weeks, there was an objective improvement with no reported side effects from the abrocitinib. At 5 months, she continued to improve and was able to discontinue acitretin. The patient’s quality of life improved significantly as did her quality and amount of sleep. At 12 months, the patient experienced substantial symptom improvement, with only one recorded flare. This was well-controlled by increasing her topical therapies. Approximately 20 months after adding abrocitinib to her treatment regimen, her physical exam had also significantly improved, revealing only post-inflammatory hyperpigmentation, no erosions, and a hypopigmented plaque, which was 95% resolved from its previous state (Figure 2). Future plans include gradually discontinuing apremilast and decreasing abrocitinib to 100 mg oral daily.

Figure 1.

Clinical images of vulvar hypertrophic and erosive lichen planus prior to initiation of abrocitinib.

Figure 2.

Clinical images of vulvar hypertrophic and erosive lichen planus at 20 months and 1 week of treatment with abrocitinib 200 mg oral daily.

Discussion

Genital lichen planus poses significant treatment challenges with substantial physical and psychological impacts on patients. ⁴ Debilitating symptoms can arise if not treated effectively, including pruritus, dysuria, dyspareunia, and burning. ⁷ Moreover, scarring resulting from poorly controlled disease can cause permanent disfiguration. Inadequate treatment raises concerns about the risk of developing squamous cell carcinomas, with the exact rates of malignant transformation remaining unknown.^7,8 Therefore, vigilant clinical monitoring to assess disease control is highly desirable. Given the extremely limited therapeutic trials for this condition, successful patient experiences, such as the present case, are vital resources for management. ⁴

First-line treatments for genital lichen planus typically include topical ultrapotent corticosteroids or calcineurin inhibitors, followed by advancing to systemic steroids, retinoids, and systemic immunosuppressants in more treatment-resistant cases. ⁴ As demonstrated in the present case, mucosal variants can be extremely treatment-resistant and require a multimodal treatment approach. Despite the pathogenesis of lichen planus not being fully understood, there have been proposals for the role of cytokines in the JAK-signal transducer and activator of transcription signaling pathway.^5,9 Prior cases of lichen planus treated with JAK inhibitors, including tofacitinib, have demonstrated efficacy and safety.^5,6 However, to our knowledge, the use of abrocitinib, a highly selective JAK 1 inhibitor, for the treatment of resistant hypertrophic and erosive vulvar lichen planus has not yet been reported.

Although abrocitinib is currently approved for atopic dermatitis, its demonstrated success in improving recalcitrant genital lichen planus as in the present case is promising and warrants further research. ¹⁰ While improvement in atopic dermatitis has been reported within 12 weeks of therapy on abrocitinib, response for genital lichen planus may take longer given its recalcitrant and sometimes hypertrophic nature. ¹⁰ The present case was extremely successful, with no reported side effects. The patient’s quality of life significantly improved leading to minimal to no itch and reduced disease flares. As it is an off-label treatment, careful patient selection and thorough discussion of potential benefits and risks are essential. Future research with large-scale studies and randomized controlled trials is needed to evaluate the safety and efficacy of abrocitinib in vulvar lichen planus.