A Report of a Case with Acrogeria

A 32-year-old female was diagnosed as Acrogeria by the following clinical characteristics; 1) atrophy of the skin with pigmentation, most marked on the backs of the hands, fingers, feet and toes, extending over the distal parts of the forearms and legs, 2) blue veins clearly visible on the trunk, 3) easy bruisability with formation of ugly scars on the lower extremities, 4) small fingernails and onychogryphosis of the nails of both big toes, 5) peculiar wrinkles arranged in semicircles around the corners of the mouth extending to the outside of the eyes. Her maternal grandparents were cousins and her mother had the same characteristic appearance as the patient. The patient had experienced the rupture of the uterus during her first labor at full term when she was 30 years old. The histological examination of the uterus showed no abnormality as the pregnant uterus and the fetus, who had died immediately after the rupture, was also found to have no abnormality. In addition to this case, 35 reported cases of Acrogeria were reviewed, and the significance of the disease in relation to the premature aging syndrome was discussed.