tofacitinib and Lichen-Planus

Lichen planus is a chronic inflammatory immune disorder that most commonly affects the skin and mucous membranes. Esophageal lichen planus (ELP) is a frequently misdiagnosed and poorly understood form of lichen planus that can be asymptomatic or present with dysphagia and odynophagia caused by the formation of erosions and strictures in the esophagus. These strictures often reduce a patient's quality of life and may lead to emaciation in more severe cases. We present the case of an 89-year-old woman with a history of cutaneous lichen planus (CLP) and mucosal lichen planus that were successfully managed with topical corticosteroids and oral cyclosporine rinses who presented with an esophageal stricture and erosions that were treated unsuccessfully with surgery. Our patient's condition continued to worsen until she presented in an emaciated state and was treated with tofacitinib, which resulted in complete resolution of oral lichen planus (OLP), ELP, and genital lichen planus.

Topics: Aged, 80 and over; Constriction, Pathologic; Esophagus; Female; Humans; Lichen Planus; Quality of Life

Topics: Esophagus; Humans; Janus Kinase 1; Lichen Planus; Piperidines; Pyrimidines

Topics: Alopecia; Female; Humans; Janus Kinase Inhibitors; Lichen Planus; Middle Aged; Nail Diseases; Piperidines; Pyrimidines; Treatment Outcome

Topics: Aged; Female; Humans; Janus Kinase Inhibitors; Keratinocytes; Lichen Planus; Male; Middle Aged; Mouth Mucosa; Piperidines; Pyrimidines; Signal Transduction; STAT Transcription Factors

Topics: Adult; Aged; Female; Humans; Lichen Planus; Male; Middle Aged; Piperidines; Pyrimidines; Retrospective Studies; Treatment Outcome

Topics: Humans; Lichen Planus; Piperidines; Pyrimidines; Pyrroles

Lichen planopilaris (LPP) is an inflammatory cicatricial alopecia for which many different therapies are attempted with varying success. The Janus kinase (JAK) inhibitor, tofacitinib, has been shown to be effective in treating the noncicatricial alopecia, alopecia areata. As in alopecia areata, upregulation of interferon and JAK signaling may play a role in LPP. We retrospectively reviewed the cases of 10 patients with recalcitrant LPP who were treated with oral tofacitinib. Patients received oral tofacitinib 5 mg twice or three times daily for 2-19 months as either monotherapy or adjunctive therapy to other ongoing treatments including intralesional triamcinolone, hydroxychloroquine, and tacrolimus ointment. Eight patients had clinical improvement in LPP with tofacitinib as either monotherapy (4/10) or adjunctive therapy (4/10). LPP Activity Index (LPPAI) before and after treatment was measured in seven patients and was significantly different (6.22 before treatment, 3.08 after treatment; p value = .0014). Reduction in LPPAI ranged from 30 to 94%. One patient complained of 10 pound (4.5 kg) weight gain after 12 months on tofacitinib. No other adverse effects were reported. Treatment with oral tofacitinib either as monotherapy or adjunctive therapy can lead to measurable improvement in recalcitrant LPP.

Topics: Administration, Oral; Adult; Aged; Alopecia; Dermatologic Agents; Drug Administration Schedule; Female; Humans; Janus Kinase Inhibitors; Lichen Planus; Male; Middle Aged; Piperidines; Pyrimidines; Pyrroles; Remission Induction; Retrospective Studies; Scalp; Scalp Dermatoses; Skin; Time Factors; Treatment Outcome

Lichen planus is a chronic inflammatory disease of the skin, mucous membranes and nails. Management of oral involvement, particularly atrophic and erosive lesions, is challenging. Noteworthy, there is a lack of published evidence for treatment. Cell-mediated cytotoxicity is actually regarded as a major mechanism of pathogenesis. The interferon-gamma induced chemokines CXCL10 and CXCL9 are strongly expressed in serum of patients as well as in both skin and mucosal lesions. Therefore the interferon gamma/CXCL10 axis is considered a key process for both progression and maintenance of chronic cytotoxic inflammation. According to these findings, the interferon gamma/CXCL10 axis could be considered a therapeutically attractive target to reverse inflammation. Since interferon gamma signal transduction occurs through JAK 1 and 2, JAK inhibitors could lead to blockade of interferon gamma signaling and downstream CXCL10 expression.

Topics: Chemokine CXCL10; Chemokine CXCL9; Enzyme Inhibitors; Humans; Inflammation; Interferon-gamma; Lichen Planus; Models, Theoretical; Piperidines; Promoter Regions, Genetic; Pyrimidines; Pyrroles; Signal Transduction; Skin; Treatment Outcome