Malik and Grzeschik (2008) reported a large consanguineous Pakistani family in which 25 individuals spanning 5 generations had lymphedema confined to the lower limbs. Despite consanguinity, detailed pedigree analysis indicated autosomal dominant inheritance with reduced penetrance. Onset occurred ... Malik and Grzeschik (2008) reported a large consanguineous Pakistani family in which 25 individuals spanning 5 generations had lymphedema confined to the lower limbs. Despite consanguinity, detailed pedigree analysis indicated autosomal dominant inheritance with reduced penetrance. Onset occurred in early childhood and showed maximum manifestations at puberty. The phenotype was variable, ranging from swelling of a foot to severe bilateral lymphedema of the lower legs below the knee. Some, but not all, patients had papillomatosis over the affected areas. Swelling began to fade away by age 30 years and became unremarkable in the following 12 to 15 years. The phenotype was similar to that seen in hereditary lymphedema type IA (153100).