HMGB1 is a non-histone nuclear protein, the prototype of the so-called damage-associated molecular pattern or alarmins [6], whose levels have been demonstrated as significantly elevated in patients affected by RDEB, in both blisters fluid and serum [7], and positively correlated to the Birmingham Epidermolysis Bullosa Severity Score [8]. This evidence concerns the gene HMGB1 and recessive dystrophic epidermolysis bullosa.