ARTERIOPATHIA CALCIFICANS INFANTUM HISTOLOGICAL AND HISTOCHEMICAL FINDINGS

An eight months old child died after a brief illness during which it showed temperature, cyanosis, and dyspnea. The autopsy revealed an arteriopathia calcificans infantum. From histological and histochemical results the following conception of the pathogenesis of the disease was deduced: Arteriopathia calcificans infantum starts with degenerative changes and iron-impregnation of the lamina elastica interna. This is followed by a concentration of acid mucopolysaccharides near and around the defect fibres. Calcium and phosphates are deposited into these mucopolysaccharides around which - in a secondary process - granulation tissue develops. This tissue destroys the muscle fibres of the tunica media and also removes calcium deposites in some places. Furthermore, a reactive endangitis is observed which leads to a stenosis and obliteration of the blood vessels. From our own observation and from those published in the literature it seems obvious that arteriopathia calcificans infantum is caused by a humoral factor which has a specific affinity for elastic fibres. © 1969 Springer-Verlag.