@entity6715 ( @entity6715 ) and @entity137 ( @entity137 ) are rare conditions characterized by extensive @entity1713 and @entity33 . Both are associated with a high mortality rate and significant long-term morbidity. Since the initial report introducing the term @entity137 in 1956, diagnosis of the condition has been fraught with difficulties that continue to exist today. The terms ' @entity9374 ' (EMM) and @entity6715 , and their relationship to @entity137 have also been confusing to clinicians. It is now recognized that EMM is a different entity from @entity6715 and @entity137 in terms of demographics, causality and severity. @entity6715 and @entity137 represent a continuum of disease, and differ only by the extent of @entity1713 and therefore severity. The term ' @entity6715 ' (EN) is used in this article to describe the spectrum of disease that includes @entity6715 and @entity137 . Important advances in understanding the pathomechanism and treatment of EN have been made over the years. These include the recognition of human leucocyte antigen (HLA) associations (e.g. @entity1530 *1502 with @entity2049 -induced @entity137 ) and understanding of the pathogenic roles of drug-specific cytotoxic T cells and granulysin. It was previously believed that widespread keratinocyte death in EN is predominantly mediated by soluble Fas-ligand and that intravenous immunoglobulin therapy is useful in blocking this mechanism with resultant survival benefits. Further studies have since proven these theories to be incorrect. This short review describes the key advances in the terminology, classification, causality and treatment of EN, and identifies future priorities and challenges in the understanding and management of this condition.

XXXX : 60  years of errors and advances.

@entity137 :: ('MESH:D064420', 'Disease') :: ['TEN', 'toxic epidermal necrolysis'] @entity6715 :: ('MESH:D013262', 'Disease') :: ['SJS', 'epidermal necrolysis', 'Stevens-Johnson syndrome'] @entity1530 :: ('3106', 'Gene') :: ['HLA-B'] @entity1713 :: ('MESH:D012163', 'Disease') :: ['epidermal detachment'] @entity33 :: ('MESH:D052016', 'Disease') :: ['mucositis'] @entity2049 :: ('MESH:D002220', 'Chemical') :: ['carbamazepine'] @entity9374 :: ('MESH:D004892', 'Disease') :: ['erythema multiforme major']
@entity6715 :: (MESH:D013262,Disease) :: ['Epidermal necrolysis']
