It is actively released in an acetylated form from activated immune cells and passively released in the nonacetylated form during necrotic cell death.8 Previous studies have shown that total HMGB1 is significantly elevated in serum from patients with drug reaction with eosinophilia and systemic symptoms (DRESS)9 and drug‐induced SJS,7 at the time of the reaction and may discriminate between serious cutaneous drug eruptions and milder phenotypes [maculopapular exanthema (MPE)]. Here, HMGB1 is linked to Schwartz-Jampel syndrome.