Emerging treatments

Dapsone

Dapsone has been reported as beneficial in persistent or recurrent EM.[48][49] ​​In one systematic review, the overall complete response rate among patients with recurrent EM treated with dapsone was 29% (9/31).[46]​ Further studies are required.​

Rituximab

Rituximab has been tried in 5 patients with refractory EM with between 9 and 20 years of disease activity.[50] Four of these patients had antidesmoplakin antibodies. Four of the patients experienced complete or quasi-complete remission, and one patient experienced partial remission, for 3 to 11 months.

Adalimumab

Adalimumab is a recombinant human IgG1 monoclonal antibody against human tumor necrosis factor. It has been successfully used to treat recurrent EM secondary to herpes simplex virus.[51] Caution must be used because adalimumab can trigger hypersensitivity reactions, including EM and Stevens-Johnson syndrome.

Apremilast

Apremilast is a phosphodiesterase-4 inhibitor. It has been successfully used to treat recurrent oral EM.[52]​ In one case series, three patients with oral EM refractory to corticosteroid and antiviral treatment experienced complete remission without recurrences for up to 6 months after treatment with apremilast.[52]

Immunosuppressive therapy and phototherapy

A few isolated case reports and case series describe use of phototherapy (oral psoralens plus ultraviolet A) or immunosuppressive therapy with mycophenolate, azathioprine, or cyclosporine.[36][53]​​[54]​ In one systematic review, 80% (12/15) of patients completely responded to treatment with azathioprine and 37% (3/8) complete responded to mycophenolate, but risk of bias in the included studies was high.[46]

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