Epidermal necrolysis (StevenseJohnson syndrome and toxic epidermal necrolysis): Historical considerations

Objective: To describe the history of toxic epidermal necrolysis, before and after the original report by the British dermatologist Alan Lyell in 1956. Methods: Subjective expert choice of key advances in the comprehension of the nosology, classification, causality, and mechanisms of epidermal necrolysis (EN) over more than a century. Results: Epidermolysis had been reported long before Lyell’s paper, but most cases had likely been staphylococcal scalded skin syndrome in children. Concerning non-Staphylococcus EN, confusion with erythema multiforme dissipated and its relation to StevenseJohnson syndrome was clarified. Tremendous advances were made in understanding the causes and mechanisms, with increased acceleration in the last 10 years. Conclusion: The next decade should be devoted to improve the prevention and management of a disease that is the most terrible form of drug hypersensitivity. Copyright 2013, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. History of the disease: Nosology/classification, and diagnosis Original description of toxic epidermal necrolysis The name “toxic epidermal necrolysis (TEN)”was introduced in 1956 by Lyell1 to describe the clinical disorder characterized by extensive destruction of epidermis that resembled scalding. Since then, the eponym Lyell’s syndrome becamewidely accepted. As acknowledged later by Lyell,2 his original paper mixed three different disorders that all had been already described more or less clearly. Thefirst onewas rapidly identified as staphylococcal scalded skin syndrome,3 and Lyell himself4 (Figure 1) contributed to the discovery of the toxin responsible for a superficial, subcorneal detachment that is histologically very distinct from the deep necrolysis characterizing TEN.5 This had been previously recognized and reported as “neonatal pemphigus”6,7 or von Rittershain disease.8 The second is now labeled as generalized bullous fixed drug eruption (GBFDE),9 a disease characterized by necrolysis of fullthickness epidermis on large well-demarcated blisters, which is similar to that of TEN; however, it is distinct from TEN in the absence or, if present, mildness of mucous membranes erosions, usual relapses, as well as with mild differences in histopathology. d’Alembert, 92160 Antony, iwanese Dermatological Associatio Present knowledge remains insufficient to define the boundaries of GBFDE versus TEN clearly. The description of TEN had already been provided by some predecessors to Lyell under different denominations: “unusual bullous eruption” (Lang andWalker in 195610), “erythrodermawith epidermolysis” (Debré et al 193911). Erythema multiforme, StevenseJohnson syndrome, and related diseases: History and confusion The initial description of erythema exudativum multiforme is attributed to von Hebra.12 I was not able to consult von Hebra’s original reports but from photographic pictures of Hebra’s drawings it seemed to encompass many skin diseases with a circinate pattern, of which some would probably be given a variety of other denominations today [e.g., erythema annulare, pyoderma gangrenosum, subacute lupus erythematosus (LE), Sweet’s syndrome]. Rendu in 1916,13 Fiessinger and Rendu in 191714 described a mucocutaneous eruption without clear cause that was later on reported as FiessingereRendu syndrome or pluriorificial erosive ectodermosis, and retrospectively seems very similar to the two cases described in 1922 by Stevens and Johnson.15 In Germany Fuchs syndrome is used to describe a variant of erythema multiforme, and this syndrome mainly involves the mucosal surfaces. As the skin may be completely unaffected, it is an underrecognized diagnosis and often difficult to confirm.16 n. Published by Elsevier Taiwan LLC. All rights reserved. Figure 1 Alan Lyell speaking at the first International Symposium on TEN, Créteil 1985. Table 1 SCAR study classification of EMM and Epidermal necrolysis. Classification Type of lesions Distribution % BSA