LYELLS-SYNDROME - TREATMENT WITH CENTOXIN AND SELENIUM

Lyell's syndrome (LS) (toxic epidermal necrolysi) is also known as ''s calded skin syndrome.'' When caused by staphylococci, it has a favorab le prognosis. However, as a drug reaction, primarily to sulfonamides, anticonvulsives, and antirheumatic agents, it often takes a fatal cour se. On the basis of a case report, the authors present a new therapeut ic concept aimed at influencing the pathobio-chemical changes underlyi ng LS. After taking a single dose of a sulfonamide preparation for a m ild urinary tract infection, a 14-year-old girl developed LS with skin changes covering almost the whole body surface. Within 12 h the child showed signs of sepsis with imminent organ failure, leuco- and thromb ocytopenia, and symptoms of beginning enteroparalysis. Whole-blood che miluminescence analyses revealed massive formation of reactive oxygen species. Parallel to this, a drastic increase in PMN-elastase and stro ng activation of the kallikrein-kinin system were found. Blood concent rations of glutathione peroxidase and reduced glutathione were extreme ly low. The therapeutic regimen was intended to prevent both endotoxin emia and overproduction of toxic oxygen metabolites: the girl received 100 mug monoclonal antibody against endotoxin (Centoxin) in combinati on with sodium selenite (Selenase). By day 2 her circulatory condition had stabilized and the blood concentration of oxygen radicals decreas ed. Neither liver nor kidney function showed pathological changes. Alt hough on day 2 maximum activation of the kallikrein-kinin system was o bserved, the expected drop in blood pressure did not occur. The blood concentration of elastase fell markedly, indicating that the release o f elastase from neutrophil granulocytes, stimulated by endotoxin and c omplement, could be prevented. Both blood and plasma activities of glu tathione peroxidase increased sharply as a consequence of the selenium therapy. On the 8th day of therapy all parameters had returned to nor mal (elastase, kallikrein-kinin, leucocytes, selenium). The whole-bloo d chemiluminescence response showed normal values and the skin changes had almost completely disappeared.