Macrodystrophia Lipomatosis:A Case Report

Macrodystrophia Lipomatosa is a rare cause of congenital macrodactyly characterized by progressive proliferation of all mesenchymal elements, with disproportionate increase in fibroadipose tissue. This anomaly is reportedly more common in the foot than in the hand with predilection for the plantar and median nerve distribution. This rare cause of gigantism of limb can be confused with other common causes like congenital lymphedema. We report a case of 2 years old female child presenting in outdoor as a painless progressive enlargement of index finger, middle finger and thumb of right hand since 6 months of age causing mechanical difficulty.