Fumaric acid esters (FAEs) have proven efficacy in the treatment of
psoriasis and have been in use for decades. More recently, as their mechanism of action and abundant immunomodulatory effects become clearer, the potential benefits of treating other inflammatory skin conditions using FAEs are increasingly being recognized. The use of FAEs as combination systemic
therapy has not been well studied and data are lacking regarding the safety and efficacy of this type of
therapy. In this case report, three patients with severe, extensive and recalcitrant cutaneous manifestations of
systemic lupus erythematosus (SLE) (one case of disseminated discoid lesions and two with severe
chilblain lesions) were treated with Fumaderm® (containing the FAE
dimethylfumarate and monoethylhydrogen
fumarate salts), after failing to respond to a multitude of other monotherapies and combination
therapies. All patients showed a substantial clinical response when FAEs were added to their treatment, with concurrent improvements in quality-of-life instrument scores. The treatment was well tolerated in the context of systemic organ involvement and as combination
therapy with other agents, such as
hydroxychloroquine and
mycophenolate mofetil. These cases of SLE illustrate the potential use of FAEs in severe, disfiguring and otherwise
therapy-resistant skin lesions, including, to our knowledge, the first two reported cases of FAE-treated
chilblain lupus erythematosus.