Frequency of Recurrence of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis

Jeffrey P. Callen, MD

Conditions that mimic SJS or TEN may be the true diagnosis when recurrences are frequent.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions that are associated with potential mortality and morbidity. These diseases are often but not always linked to drug intake. It is generally believed that if the offending drug or one of its close relatives is avoided, recurrence is unlikely. Generalized bullous fixed drug eruption (GBFDE) may mimic SJS/TEN (NEJM JW Dermatol May 10 2013; Br J Dermatol 2013; 168:726), but its pathogenesis is suggested to differ (J Am Acad Dermatol 2014; 70:539). Fixed drug eruptions are known to recur frequently.

These authors followed enrollees in a population-based cohort study hospitalized for a first episode of SJS or TEN between April 2002 and March 2011. In all, 567 patients had SJS (80%) and 141 had TEN; 127 were younger than 18 years. Of these, 42 (7.2%) were subsequently hospitalized for an SJS or TEN recurrence, and 8 (1.4%) experienced multiple recurrences (incidence rate, 16 episodes per 1000 person-years; median time to recurrence, 315 days). Recurrence was not analyzed by outcomes or drug data. The authors note an observed rate of SJS/TEN in the study population at least 1000-fold greater than estimated rates in the general population (1 to 7 per million person-years), perhaps reflecting a known genetic predisposition to severe cutaneous drug reactions in patients who develop SJS/TEN.

CITATION(S):

Finkelstein Y et al. Recurrence and mortality following severe cutaneous adverse reactions. JAMA 2014 Jun 4; 311:2231.