Journal
REVUE FRANCAISE D ALLERGOLOGIE
Volume 57, Issue 6, Pages 418-425Publisher
ELSEVIER MASSON, CORPORATION OFFICE
DOI: 10.1016/j.reval.2017.06.002
Keywords
Toxiderma; Toxic epidermal necrolysis; Stevens-Johnson syndrome; Maculo-papular exanthema; Cytotoxic T lymphocyte
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Toxic epidermal necrolysis is a rare and severe cutaneous adverse drug reaction. The histologic hallmark of this reaction is necrosis with detachment of the epidermis resulting in skin blistering. This is a life-threatening emergency with up to 30 % deaths in the acute phase. The percentage of blistering skin determines two clinical entities: Stevens-Johnson syndrome, in which detachment is less than 10 %, and Lyell's syndrome, in which it is greater than 30 %. The development of late complications, notably mucous synechiae, is frequent and must be systematically prevented. The pathophysiologic mechanism consists of a delayed drug hypersensitivity reaction in which cytotoxic T-lymphocytes play a major role. Genetic background is also very important with HLA-drug associations having been reported, notably in Asian populations. Treatment remains highly debated, with systemic steroids, cyclosporine or intravenous immunoglobulins all being commonly used. Some studies also suggest that TNF-alpha inhibitors are of value in treating this disease. (C) 2017 Elsevier Masson SAS. All rights reserved.
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