Alpha1-antitrypsin deficiency and toxic shock: a Japanese autopsy case

A 74-year-old Japanese female presented with the sudden appearance of hemorrhagic purpuric ecchymoses on her lower extremities and with fever and chills, and died on the fifth day of hospitalization. A diagnosis of alpha1-antitrypsin (AT) deficiency was made postmortem. The liver weighed 1260 g. Histological sections from the liver revealed rather severe fatty changes of the hepatocytic parenchyma and partial loss of the normal hepatic architecture with fibrosis. The hepatocytes contained periodic acid-Schiff (PAS)-positive, diastase-resistant and alpha1-AT-positive intracytoplasmic globules. There was markedly increased inflammatory infiltration with severe edema and congestion, accompanied by fibrous, thickened pulmonary alveolar walls with fibrin deposition in the lungs (right, 410 g; left, 280 g), which suggest findings similar to those seen in multiple organ failure. Mild pulmonary emphysema was also present in the upper lobes of the lungs. Histological sections from the hemorrhagic necrotic ecchymoses of the skin showed marked neutrophil infiltration over the subcutaneous tissue with bleeding and blistering. A finding of thrombophlebitis was also found in the subcutaneous tissue. No bacteria were detected in the ecchymoses, the urine or the blood. Plasma protein analysis revealed a lower level (9.5 micromol/L) of alpha1-AT and a higher level (330 U) of anti-streptolysin O (ASO). These findings suggest that the patient died of toxic shock-like syndrome and that alpha1-AT deficiency might have facilitated the development of the toxic shock. To our knowledge, this is the first case of toxic shock associated with alpha1-AT deficiency.