%0 Journal Article
%T Rapidly Developing Toxic Epidermal Necrolysis
%A Viktoria Oline Barrios Poulsen
%A Jonas Nielsen
%A Troels Dirch Poulsen
%J Case Reports in Emergency Medicine
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/985951
%X Severe cutaneous reactions with potentially fatal outcomes can have many different causes. The Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare. They are characterized by a low incidence but high mortality, and drugs are most commonly implicated. Urgent active therapy is required. Prompt recognition and withdrawal of suspect drug and rapid intervention can result in favourable outcome. No further international guidelines for treatment exist, and much of the treatment relies on old or experimental concepts with no scientific evidence. We report on a 54-year-old man experiencing rapidly developing drug-induced severe TEN and presented multiorgan failure involving the respiratory and circulatory system, coagulopathy, and renal insufficiency. Detachment counted 30% of total body surface area (TBSA). SCORTEN£¿=£¿5, indicating a mortality rate >90%. The patient was sedated and mechanically ventilated, supported with fluids and inotropes to maintain a stable circulation. Component therapy was guided by thromboelastography (TEG). The patient received plasmapheresis, and shock reversal treatment was initiated. He was transferred to a specialized intensive care burn unit within 24 hours from admittance. The initial care was continued, and hemodialysis was started. Pulmonary, circulatory, and renal sequelae resolved with intensive care, and re-epithelialization progressed slowly. The patient was discharged home on hospital day 19. 1. Introduction The Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but present severe skin manifestations. They are estimated to occur in 1¨C3 people/million/year in Europe and the USA [1, 2]. They are characterized by a low incidence but high mortality, and drugs are most commonly implicated in 80% of TEN cases [3, 4]. TEN is the most severe form of drug-induced skin reaction and is defined as epidermal detachment of >30% of total body surface area (TBSA). SJS presents with epidermal detachment of <10% of TBSA, whereas involvement of 10%¨C30% of TBSA is defined as SJS/TEN overlap [1, 2, 5]. Even in extensive disease, the hairy portion of the scalp is generally not affected [5]. The pathomechanism of drug-induced SJS/TEN is not completely understood but is assumed to be an immune-mediated skin reaction involving CD8 T cells, cytotoxic reactions, and delayed hypersensitivity [1, 6]. Histopathology is characterized by keratinocyte apoptosis followed by necrosis, which creates the basis for the pronounced epidermal erosion and detachment. There is a common agreement to consider this
%U http://www.hindawi.com/journals/criem/2013/985951/