Toxic epidermal necrolysis data from the CELESTE multinational registry. Part I: Epidemiology and general...

Toxic epidermal necrolysis (TEN) is a rare, life-threatening autoimmune disease predominantly manifested in the skin and mucous membranes. Today, infectious complications have the dominant share in mortality of TEN patients. Due to the nature of the therapy and administration of immunosuppressive medications, a wide range of potentially pathogenic microorganisms, which cause infectious complications in different compartments in these patients, is not surprising.

This is a multicentric study, which included all patients with TEN hospitalized between 2000-2015 in specialized centres in the Czech Republic and Slovakia. The total catchment area was over 12.5 million inhabitants. The actual implementation of the project was carried out using data obtained from the registry CELESTE (Central European LyEll Syndrome: Therapeutic Evaluation), when specific parameters relating to epidemiological indicators and infectious complications in patients with TEN were evaluated in the form of a retrospective analysis.

In total, 39 patients with TEN were included in the study (12 patients died, mortality was 31%), who were hospitalized in the monitored period. The median age of patients in the group was 63 years (the range was 4-83 years, the mean was 51 years), the median of the exfoliated area was 70% TBSA (total body surface area) (range 30-100%, mean 67%). SCORTEN was calculated for 38 patients on the day of admission. Its median in all patients was 3 (range 1-6; mean 3). Any kind of infectious complication in the study group was recorded in 33 patients in total (85%). In total, 30 patients (77%) were infected with gram-positive cocci, 27 patients (69%) with gram-negative rods, and yeast cells or fibrous sponge were cultivated in 12 patients (31%). A total of 32 patients (82%) were found to have infectious complications in the exfoliated area, 15 patients (39%) had lower respiratory tract infections, 18 patients (46%) urinary tract infections and 15 patients (39%) an infection in the bloodstream. The most common potentially pathogenic microorganism isolated in our study group was coagulase neg. Staphylococcus, which caused infectious complications in 24 patients. Enterococcus faecalis/faecium (19 patients), Pseudomonas aeruginosa (17 patients), Staphylococcus aureus (11 patients) and Escherichia coli (11 patients) were other most frequently isolated micro-organisms.

The published data were obtained from the unique registry of TEN patients in Central Europe. In the first part, we have succeeded in defining the basic epidemiological indicators in the group of patients anonymously included in this registry. The study clearly confirms that infectious complications currently play an essential role in TEN patients, often limiting the chances of survival. The study also shows a high prevalence of these complications in the period after 15days from the start of hospitalization, when most patients already have completely regenerated skin cover.