Familial necrobiosis lipoidica not associated with diabetes

A 42-year-old woman presented with a 12-year history of extensive yellow and erythematous plaques, round and oblong with irregular configuration and glossy atrophic central areas on the pretibial aspects of both legs. Her 45-year-old sister presented with a 7-year history of a single plaque with erythematous margins, abundant telangiectases, and an atrophic center in the lower portion of the left leg. There was no family history of type-1 or type-2 diabetes mellitus. Both patients had normal fasting glucose concentration, oral glucose tolerance test, and glucose overload test. Different treatment options including topical corticosteroids were unsuccessful. Treatment with oral fumaric acid esters was attempted but the medication was discontinued because of intolerable side effects (flushing and gastrointestinal discomfort). At present, after a follow-up of 2 years, the plaques remain unchanged. These two cases should be added to the few cases of familial nondiabetic necrobiosis lipoidica previously reported.