diagnostic and therapeutic challenges in erythema elevatum diutinum

Introduction: Erythema elevatum diutinum (EED) is a rare form of cutaneous fibrosing type of leukocytoclastic vasculitis leading sometimes to nodule formation, imitating neoplastic process. Case report: We present the case of a 65-year-old woman with a 6-year history of cutaneous presentation of erythema elevatum diutinum, coexisting with myasthenia gravis and monoclonal IgA gammopathy. Dermoscopy of the skin lesions showed prominent thick arborizing vessels in a concentric alignment. Previous treatment with the use of methotrexate, dapsone, mycophenolate mofetil and cyclosporine did not stop the disease progression. Laboratory tests showed the presence of monoclonal protein IgAk in the serum and X-ray examination showed numerous fine-spot thinning of bone structure, especially within the skull bones. Hematological treatment with thalidomide and cyclophosphamide was introduced. A rapid resolution of skin lesions was observed. Conclusions: This case shows that management of the underlying hematological abnormality may be essential for successful therapy of the otherwise treatment resistant erythema elevatum diutinum.