Acrogeria (Gottron type): a vascular disorder?

We report a 27-year-old Japanese man with the peculiar clinical features of acrogeria. He had had perniosis since early childhood. Prominent atrophic skin changes over the hands, hallux valgus, shortened distal phalanges and atrophic scars on his auricles were noted. X-ray of the hands revealed acro-osteolytic changes of the distal phalanges, and arteriography demonstrated multiple occluded branches of the digital arteries. There were no histological changes of systemic sclerosis in his forearm skin, nor antinuclear antibodies or coagulation disorders. Western immunoblotting demonstrated decreased production of type III collagen by dermal fibroblasts both from an affected finger and from the unaffected upper arm. Although the pathogenesis of acrogeria is unknown, the present case suggests that peripheral circulatory disturbance, as well as a congenital abnormality in type III collagen synthesis, may partly account for the pathogenesis of Gottron-type acrogeria.