Appeal type: Experimental/Investigational
Decision: Overturned

Diagnosis: Lichen planopilaris. 
Treatment: Xeljanz 10 milligram (mg) tablet. 
The insurer denied coverage for the Xeljanz 10 mg tablet. 
The denial is overturned. 

This is a patient diagnosed with lichen planus and lichen planopilaris (LP). The patient has tried and failed tacrolimus ointment, Plaquenil, and steroids in oral, topical, and intralesional forms over a period of six months. All of these medications have failed to control the patient's lichen planus and lichen planopilaris. The patient's lichen planus and lichen planopilaris have worsened per visit notes. Per the visit notes, the patient has lost most of their eyebrows and their lateral beard, and has developed a rash consistent with eruptive lichen planus on the arms and legs. The treating provider has requested therapy with Xeljanz 10 milligrams (mg) by mouth (PO) twice daily (BID), which is under review. 

It is important to understand that lichen planus and lichen planopilaris are rare conditions. Less than one-half of one percent of the population of the United States carries a diagnosis of lichen planus. Lichen planopilaris is even rarer. There are no medications specifically approved by the Food and Drug Administration (FDA) for the treatment of either lichen planus or lichen planopilaris. When treating a rare condition, for which there is no FDA-approved treatment, it is standard of care in dermatology to use medications off-label. Otherwise, dermatologists would have no therapeutic options for their patients with uncommon conditions. 

The medications are carefully chosen for use, based on the pathogenesis of the condition and the specific mechanism of action of the medication. Lichen planus and lichen planopilaris are inflammatory skin conditions, and therefore, anti-inflammatory medications are used as treatment, based on the standards of care in dermatology. As new anti-inflammatory medications become available, they are added to the standard dermatologic treatment algorithm for the treatment of lichen planus and lichen planopilaris. Xeljanz, and other medications, which have the same mechanism of action -- Janus kinase inhibitors (JAK inhibitors) -- are the newest anti-inflammatory medications to be added to the dermatological treatment guidelines for inflammatory conditions. 

There are no increased adverse risks over the standard treatments for severe lichen planus and lichen planopilaris, such as the patient is experiencing. 

The patient has already been treated with the standard first line therapies for lichen planus and lichen planopilaris (topical steroids and a topical calcineurin inhibitor), as well as receiving multiple second line therapies (oral and injected steroids and Plaquenil). Despite treatment with these medications, the patient's condition has worsened and flared. The standard treatments are not at all beneficial for this patient. 

Based on the demonstrated efficacy and the safety of Xeljanz, this medication is the best available treatment in this clinical situation. As such, it is not reasonable to deny the use of Xeljanz in this clinical situation based on lack of FDA approval, since all of the medications used for lichen planus and lichen planopilaris lack FDA approval. 

Therefore, the request for therapy with Xeljanz 10 mg PO BID would be appropriate for use in this patient. 

In summary, the denial of Xeljanz 10 mg tablet for a date yet to be determined and filled at a network specialty pharmacy and coverage for the requested treatment should be reversed. This treatment is likely to be more beneficial than any standard treatments for this patient's condition and the adverse risks are not substantially increased over standard treatments.