Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights Save
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are among the most severe drug reactions, as they come with substantial mortality and morbidity. A review of European centers dealing with SJS and TEN shows that despite best treatment practices, the 6-week mortality rate was 21%.
This retrospective review of 13 referral centers belonging to the ToxiTEN ERN-skin subgroup identified 212 adults with SJS/TEN between 2015 and 2019. They assess offending agents, treatments and outcomes.
Among SJS/TEN patients the mean body surface area detachment was 27% and primary culprit drugs were lead by antibiotics (21.2%), then anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%).
Treatment approaches included:
- Supportive care only (38.2%)
- Systemic glucocorticoids (35.4%)
- Intravenous immunoglobulins (23.6%)
- Cyclosporine (10.4%)
- Tumor necrosis factor agents (3.3%).
Nearly two-thirds (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Acute complications was linked to body surface area detachment ≥30% (OR 2.49) and a SCORTEN greater than or equal to 2 was significantly mortality (OR, 10.30; P < .001).
Cyclosporine was associated with a >20% increase in body surface area detachment and an increased risk of infections (adjOR, 7.16). Glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40). Clearly the most severe patients received the most potent treatments.
The authors called for prospective therapeutic studies and registries to be developed.
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Anecdotally, I found that Tofacitinib 10mg bd worked fast and very well in some patients (targeting IL15 and other cytokines), while others do better with TNFi
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