A Case of DiHSS/DRESS Syndrome-Related Acute Hepatic Failure

Abstract

‘Drug Induced Hypersensitivity Syndrome’ (DiHS) or ‘Drug Rash with Eosinophilia and Systemic Symptoms’ (DRESS) syndrome is a life threatening, delayed type drug hypersensitivity reaction. This syndrome is characterized by fever, skin rash, lymphadenopathy, hematological abnormalities and visceral involvement and liver is the most frequently involved visceral organ. Liver involvement is mostly presented as acute anicteric hepatitis with elevated liver enzymes. Rarely, it can be presented as cholestasis which indicates a worse prognosis. In this article, a case of valproic acid induced-DRESS syndrome who presented with acute hepatic failure is presented. Diagnosis of DRESS syndrome may delay due to the long interval between drug intake and the onset of symptoms. The variety of symptoms can also be challenging. Early diagnosis is important in terms of reducing morbidity and mortality.

Keywords

• Drug Induced Hypersensitivity Syndrome (DiHS)
• drug allergy
• hepatic failure
• DRESS

Akut Hepatik Yetmezliğin Eşlik Ettiği DiHSS/ DRESS Sendromu Olgusu

Abstract

“Drug Induced Hypersensitivity Syndrome” (DiHS) ya da diğer adı ile “Drug Rash with Eoshinophilia and Systemic Symptoms” (DRESS) sendromu, yaşamı tehdit edebilen gecikmiş tip ilaç hipersensitivite reaksiyonudur. Ateş, deri döküntüsü, lenfadenopati, hematolojik anormallikler ve çoklu organ tutulumu ile karakterizedir. Karaciğer en sık tutulan organ olup, çoğunlukla karaciğer enzim yüksekliği ve akut anikterik hepatit şeklinde karşımıza çıkmaktadır. Çok daha nadir olarak kolestaz eşlik edebilir ve bu durum kötü prognoza işaret etmektedir. Bu yazıda valproik asit ilişkili hepatik yetmezlik ve ağır kolestazın görüldüğü DRESS sendromlu bir olgu sunulmuştur. İlaç kullanımı ile semptomların başlangıcı arasındaki sürenin uzun olması ve semptomların çeşitliliği nedeniyle DRESS sendromunun tanısında gecikmeler olabilmektedir. Hastalığın erken tanınması, morbidite ve mortaliteyi azaltmak açısından oldukça önem taşımaktadır.

Keywords

• Drug Induced Hypersensitivity Syndrome (DiHS)
• ilaç alerjisi
• hepatik yetmezlik
• DRESS

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