ISSN:
1437-9813
Keywords:
Lyell's syndrome
;
Multiple organ failure
;
Sepsis
;
Endotoxin
;
Oxygen free radicals
;
Selenium, immunotherapy
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Lyell's syndrome (LS) (toxic epidermal necrolysis) is also known as “scalded skin syndrome.” When caused by staphylococci, it has a favorable prognosis. However, as a drug reaction, primarily to sulfonamides, anticonvulsives, and antirheumatic agents, it often takes a fatal course. On the basis of a case report, the authors present a new therapeutic concept aimed at influencing the pathobiochemical changes underlying LS. After taking a single dose of a sulfonamide preparation for a mild 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 thrombocytopenia, and symptoms of beginning enteroparalysis. Wholeblood chemiluminescence analyses revealed massive formation of reactive oxygen species. Parallel to this, a drastic increase in PMN-elastase and strong activation of the kallikrein-kinin system were found. Blood concentrations of glutathione peroxidase and reduced glutathione were extremely low. The therapeutic regimen was intended to prevent both endotoxinemia and overproduction of toxic oxygen metabolites: the girl received 100 μg monoclonal antibody against endotoxin (Centoxin) in combination with sodium selenite (Selenase). By day 2 her circulatory condition had stabilized and the blood concentration of oxygen radicals decreased. Neither liver nor kidney function showed pathological changes. Although on day 2 maximum activation of the kallikreinkinin system was observed, the expected drop in blood pressure did not occur. The blood concentration of elastate fell markedly, indicating that the release of elastase from neutrophil granulocytes, stimulated by endotoxin and complement, could be prevented. Both blood and plasma activities of glutathione peroxidase increased sharply as a consequence of the selenium therapy. On the 8th day of therapy all parameters had returned to normal (elastase, kallikrein-kinin, leucocytes, selenium). The whole-blood chemiliminescence response showed normal values and the skin changes had almost completely disappeared.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00832264
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