Results
A total of 13 patients received etanercept. The mean SCORTEN was 2.2. The observed mortality was 0%, which was markedly lower than the predicted mortality of 24.3% (as determined by linear interpolation). Of this cohort, 9 patients received etanercept alone (mean SCORTEN of 2.1, predicted mortality of 22.9%), whereas 4 patients received a combination of etanercept and IVIG (mean SCORTEN of 2.3, predicted mortality of 27.2%).
The 4 patients who received both etanercept and IVIG received dual therapy for varying reasons. In patient 2 (Table 1), the perceived severity of this case ultimately led to the decision to start IVIG in addition to etanercept, resulting in rapid recovery and discharge after only 1 week of hospitalization. Intravenous immunoglobulin also was given in patient 3 (SCORTEN of 4) and patient 6 (SCORTEN of 2) for progression of disease despite administration of etanercept, with subsequent cessation of progression after the addition of the second agent (IVIG). Patient 12 might have done well on etanercept monotherapy but was administered IVIG as a precautionary measure because of hospital treatment algorithms.
Nine patients did not receive etanercept. Of this group, 5 received IVIG and 4 were managed with supportive care alone. The average SCORTEN for this group was 2.4, only slightly higher than the group that received etanercept (Table 2). The mortality rate in this group was 33%, which was higher than the predicted mortality of 28.1%.
Re-epithelialization data were available for 8 patients who received etanercept. The average time to re-epithelialization for these patients was 8.9 days and ranged from 3 to 19 days. Of these patients, 2 received both IVIG and etanercept, with an average time to re-epithelialization of 13 days. For the 6 patients who received etanercept alone, the average time to re-epithelialization was 7.5 days. Re-epithelialization data were not available for any of the patients who received only IVIG or supportive care but to our recollection ranged from 14 to 21 days.
The clinical course of the 13 patients after the administration of a single dose of etanercept was remarkable, as there was complete absence of mortality and an increase in speed of recovery in most patients receiving this intervention (time to re-epithelialization, 3–19 days). We also observed another interesting trend from our patients treated with etanercept, which was the suggestion that treatment with etanercept may be less effective if IVIG and/or steroids are given prior to etanercept; likewise, treatment is more effective when etanercept is given quickly. For patients 1, 4, 5, 7, 9, and 11 (as shown in Table 1), no prior IVIG therapy or other immunosuppressive therapy had been given before etanercept was administered. In these 6 patients, the average time to re-epithelialization after etanercept administration was 7.5 days; average time to re-epithelialization, unfortunately, is not available for the patients who were not treated with etanercept. In addition, as shown in the Figure, it was noted in some patients that the depth of denudation was markedly more superficial than what would typically be clinically observed with TEN after administration of other immunomodulatory therapies such as IVIG or prednisone or with supportive care alone. In these 2 patients with superficial desquamation—patients 7 and 9—etanercept notably was given within 6 hours of onset of skin pain.
A, Dusky erythema covering 80% of the patient’s body surface area, suggestive of incipient full-thickness epidermal necrosis, 1 hour prior to etanercept administration (patient 4). B, Superficial desquamation mimicking sunburn 7 days after etanercept administration.