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Fumaric acid esters in severe ulcerative necrobiosis lipoidica: a case report and evaluation of current therapies.

Necrobiosis lipoidica (NL) was first described by Oppenheim in 1929. NL is a granulomatous skin disease with association with diabetes mellitus (DM) in 10–40% of cases, but less than 1% of patients with DM develop NL. Additional risk factors for NL include other gra-nulomatous diseases, such as granuloma anulare (GA), sarcoidosis, colitis ulcerosa and Crohn’s disease. Middle-aged women are mainly affected. The lesions typically present as irregular or ovoid plaques with a central yellow atrophic area and a violaceous border; telangiectasia is often present. Ulcerations occur in approximately one-third of patients and complicate the clinical course. The most common sites of NL manifestation are the lower legs. Topical glucocorticosteroids represent the standard treatment of NL. In more complicated cases, glucocortico-steroids are given systemically. Several other therapies, including phototherapy, as well as systemic treatment with antimalarial drugs, cyclosporine and tumour necro-sis factor (TNF) antagonists have been reported (Table I). However, a constantly effective and safe treatment has not yet been established. Especially in patients with steroid-refractory severe ulcerative NL, safe and effective therapies are needed. We report here the first case of a young woman with long-standing ulcerative NL who did not respond to topical and systemic glucocorticosteroids or phototherapy, but was treated successfully with fuma-ric acid esters (FAE).CASE REPORT