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In many respects, lipedema of the arms and legs is still an underresearched disease within the lymphatic spectrum. It is clear that clinical symptoms frequently include symmetrical fat distribution in the arms and legs and pathognomonic tenderness in female family members. However, 75 years after the first descriptions provided by Allen and Hines, we still lack pathological evidence that would provide more insight than that offered by the theses proposed by Marsch and Brauer. We also lack information about hormonal influence on hyperplastic fatty tissue and the causes of obviously increased lymph formation in the fatty tissue in patients with lipohyperplasia dolorosa. Much more is known about the effects of combined decongestive therapy, which has been used since the 1960s. Moreover, since 1997, surgery has been used to successfully treat this disease presentation. The success rate in long-term observation (15 years) is 97%.