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Lyell, a lethal syndrome

Author: 
Gonçalo Sarmento, Beatriz Frutuoso, Andreia Seixas, Tiago Teixeira, Margarida Mota
Subject Area: 
Health Sciences
Abstract: 

The Lyell Syndrome is a severe toxicodermy, rare, characterized by fever, cutaneous necrosis, and epidermal desquamation in over 30% of the body, including mucositis. It is a late anaphylactic reaction caused mainly by pharmacies. The authors present a case of a woman, aged 22, VIH positive at A1 stage, medicated during 5 days, the 15 days before admission, with carbamazepine (CMZ) for a trigeminal neuralgia, admitted to the emergency room (ER) with fever (40ºc) and a generalized cutaneous rash including hand palm, oral, genital and eye mucous membranes. Blood cultures and serology were negative. The rash kept evaluating despite the beginning of corticotherapy, with the appearance of skin detachment, Nikolsky sign and uncontrolled pain. At the 5th day of admission, the patient entered in Toxic Shock, with multiorgan dysfunction, and was transferred to a Burn Unit (IBU), where she died with a septic shock caused by Acinetobacter baumanni bactaeriemia.

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