Macrodactyly and the epidermal nevus syndrome

Macrodactyly is an uncommon congenital anomaly that affects the fingers and toes. Speculation as to cause is focused on the association with connecctive tissue abnormalities, such as neurofibromatosis. We report a highly unusual case of a patient with epidermal nevus syndrome, a specific connective tissue and skeletal disease, who also exhibited bilateral, four finger macrodactyly. Potential causes of linkage between these two specific and unusual syndromes are discussed. Treatment of the epidermal nevus may be nonoperative, differing from the philosophy of aggressive treatment of the similarly appearing premalignant congenital hairy nevus.