2022, Number 2
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Alerg Asma Inmunol Pediatr 2022; 31 (2)
Carbamazepine-induced DRESS syndrome: a severe delayed hypersensitivity reaction
Bonilla-Bustos AB, Moribe-Quintero IC, Gómez-Urrego JF
Language: Spanish
References: 33
Page: 51-57
PDF size: 226.38 Kb.
ABSTRACT
Drug reaction syndrome with eosinophilia and systemic symptoms (DRESS) is classified as a severe and potentially life-threatening type IVb hypersensitivity reaction, which occurs after exposure to drugs mainly aromatic anticonvulsants, allopurinol and some antimicrobial agents. Its incidence in children is not really known, but is estimated at 1/1,000-10,000 patients exposed to the drug. Mortality is documented at approximately 10%. The precise pathogenesis of DRESS remains unclear, however, in recent years it has been postulated that it is the result of the interaction of the following factors: pharmacogenetic, infection or reactivation of certain viruses, mainly human herpes virus 6 and 7, cytomegalovirus (CMV) and Epstein-Barr (EBV) and immunological. The classic clinical presentation includes fever preceded by a morbilliform rash, hematological alterations and internal organ involvement. The most common visceral organ involved is the liver. The onset of symptoms is usually delayed, ranging from two to eight weeks after initiation of treatment with the triggering drug. Immediate discontinuation of the offending drug and initiation of corticosteroids are the mainstay of treatment. We describe the clinical case of a 14-year-old female patient with carbamazepine-induced DRESS syndrome evaluated with the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) as a "probable case" with difficulty in establishing the diagnosis due to the heterogeneity of the clinical manifestations observed; once confirmation was achieved, the evolution was satisfactory.
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