BACKGROUND
Lichen planus is a chronic, mucocutaneous disease of unknown etiology that has no definitive cure.1,2 It is characterized by nonspecific inflammation, commonly affecting the oral and genital mucosa, nails, skin, and scalp.2,3 Oral lichen planus (OLP) occurs more frequently than the cutaneous form and is the most common cutaneous disease of the oral mucosa.2,3 OLP tends to be more persistent and resistant to therapy, posing a management challenge to dentists and dermatologists.1,2 OLP presents clinically as reticular, erosive, or ulcerative lesions with whitish streaks, producing pain and sensitivity to spicy foods.4
Although high quality evidence for the treatment of lichen planus is sparse,1 management typically involves topical corticosteroids, immunomodulators, and retinoids, as well as systemic hydroxychloroquine, methotrexate, thalidomide, and griseofulvin.2 Apremilast is an oral phosphodiesterase 4 (PDE4) inhibitor recently approved for the treatment of moderate-to-severe plaque psoriasis, a condition that involves a cell-mediated immune response with some similarities to OLP.5
CASE PRESENTATION 1
A 73-year-old female presented with a 12-year history of open mouth sores that made eating uncomfortable. Her condition was initially identified in 2002 by her dentist, who suspected OLP, and she was referred to a maxillofacial specialist. She was told to avoid citrus and spicy foods and was prescribed lidocaine 2% solution to alleviate the burning sensation. A biopsy of the oral mucosa performed in 2003 confirmed the diagnosis of OLP, with no evidence of malignancy. Topical treatments, including corticosteroids, mouthwash formulations, and tacrolimus ointment, provided little relief.
In 2006, the patient was prescribed hydroxychloroquine 200 mg twice daily, with topical tacrolimus 0.1% as needed for flares. She continued to experience frequent flares over the next 1.5 years
before discontinuing the oral therapy due to lack of efficacy, the need for regular blood monitoring, and safety concerns. In 2009, she was prescribed methotrexate, which partially controlled her condition over the next year, but she still experienced periodic flares and difficulty with certain foods. Additional treatment options for her recalcitrant condition were discussed, including tumor necrosis factor inhibitors. However, due to problems with insurance coverage for biologic agents, she was prescribed the immunosuppressant mycophenolate mofetil, at 500 mg twice daily to start, and 2 g daily by the second month. After 8 months, she once again discontinued oral therapy due to lack of efficacy and the need for regular blood tests. She maintained treatment with topical dapsone gel and topical corticosteroids, although with limited benefit, for the next 10 months.
When the patient returned to my clinic in early 2015, she had oral mucosal blisters, inflammation, pain, and difficulty eating (Figure 1). She was given a 2-week supply of apremilast and instructed to follow the 5-day titration schedule, to reach a dose of 30 mg twice daily. At the 2-week follow-up visit, the patient reported that she was free of pain and open sores, and was able to eat whatever she desired for the first time in a very long time. In her words, her mouth had “never felt so good.†After 1 month of apremilast therapy, the patient experienced a mild flare, for which an oral prednisone (40 mg) taper was added while she maintained the apremilast therapy. Two months later, a short course of prednisone (20 mg for 3 days) was added to control another mild flare (Figure 2). The patient has now been taking apremilast 30 mg twice daily for more than 5 months and is very satisfied with the control of her condition. Her mouth sores have healed, and she continues to take a low dose of prednisone (5 mg daily) to prevent flares (Figure 3). She is able to eat citrus and spicy foods without restriction, and appreciates that no blood monitoring is required. She has not experienced any side effects that were observed in clinical trials of apremilast, such as diarrhea, headaches, or depression.