Patients with Stevens Johnson syndrome and Toxic Epidermal Necrolysis Develop Coagulopathies Similar to Those Seen in Burn Patients: A Pilot Study

Abstract

Areta Kowal-Vern, Jeanine M. Walenga, Debra Hoppensteadt and Richard L.Gamelli

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a continuum of a life-threatening skin loss condition due to an immune or hypersensitivity reaction. Patients are frequently treated in burn centers.

Objective: This study was undertaken to determine if patients with SJS and TEN have a coagulopathy with comparable hemostatic perturbations to those seen in patients with burn injury.

Materials & Methods: Blood plasma parameters studied were factors of coagulation, fibrinolysis, Interleukin-6 (IL-6) and Endothelin-1. Results were compared to historical hemostatic and cytokine data from burn patients treated at the same center.

Results: Sixteen patients with SJS-TEN (6 males/10 females) with ≥20 % total body surface area (TBSA) sloughed skin were studied. The majority had received phenytoin or an antibiotic as the precipitating medication for the SJS-TEN. There was a significant increase in Thrombin-Antithrombin Complex (TAT) p<0.0004, tissue Plasminogen Activator (tPA), p<0.02, Plasminogen Activator Inhibitor-1 (PAI-1), p<0.02, and D-dimer p<0.007 plasma levels on admission. Antithrombin (AT), p<0.04 and plasminogen (PLG) p<0.02 plasma levels were significantly decreased. Conventional global coagulation tests (prothrombin and partial thromboplastin times) were not abnormal in patients with ≤7 days duration of the rash on admission. Patients with delayed admission at >7 days after the start of the rash had a significantly increased chance of demise, p<0.01. These patients also had a significantly decreased AT levels (p<0.01) compared to normal controls and to patients admitted at ≤7 days of the disease process, (p<0.01). The pattern of hemostatic aberrations of TAT, tPA, PAI1, D-dimer, Interleukin -6, AT, and PLG was similar to that seen in burn patients during the acute phase of injury and resuscitation. The mortality rate was 37.5 %.

Conclusions: Patients with ≥20% TBSA SJS-TEN had hemostatic perturbations consistent with those observed in ≥20% TBSA burn injuries coagulopathies.

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