Myopathic form of arthrogryposis and microcirculation lesion

A microvascular lesion characterized by extensive platelet aggregation, thrombosis, vascular damage with hemorrhages was found in the muscle of a 2-month-old boy with a myopathic form of the arthrogryposis syndrome. The lesion morphologically resembled the vascular leakage seen in immunologically mediated tissue injury. A degradative effect of proteases released during platelet and neutrophil aggregation on the muscle and joints is suggested.