Karbamazepine Bağlı Dress Sendromu; Nadir Görülen Bir İlaç Hipersensitivite Reaksiyonu

Öz

DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) sendromu, ateş, deri döküntüsü, lenfadenopati ve iç organ tutulumuyla karakterize, yaşamı tehdit edebilen ve çocukluk çağında nadir görülen bir gecikmiş tip ilaç hipersensitivite reaksiyonudur.Beş yaşında kız hasta, epilepsi tanısı ile karbamazepin tedavisi başlandıktan bir ay sonra, vücudunda yaygın döküntü ve ateş yakınmalarıyla başvurdu. Fizik muayenede ateş, deride yaygın makülopapüler döküntü, submandibular bölgede bilateral lenfadenopati ve hepatomegali mevcuttu.Hastaya klinik bulgular ve laboratuvar incelemeleri (atipik lenfositlerin varlığı, eozinofili ve hipertransaminazemi) sonucunda DRESS sendromu tanısı konuldu ve destek tedavisi, sistemik kortikosteroid ve intravenöz immünglobulin (İVİG) ile tedavi edildi.İlaç kullanma öyküsü, ateş, deri döküntüsü, lenfadenopati ve iç organ tutulumu bulguları olan çocuklarda ayırıcı tanıda DRESS sendromu göz önünde tutulmalıdır. Tedavide temel prensipler, hastalığa erken tanı konulması, neden olduğu düşünülen ilacın kesilmesi ve destek tedavisidir. Özellikle ciddi olgularda sistemik kortikosteroid ve İVİG tedavisi kullanılması önerilmektedir.

Anahtar Kelimeler

• Antikonvülzan, Çocuk, DRESS sendromu, Hipersensitivite, Karbamazepin

Carbamazepine-Related Dress Syndrome; A Rarely Seen Drug Hypersensitivity Reaction

Öz

The DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome, characterized by fever, skin rash, lymphadenopathy and internal organ involvement is a delayed-type drug-hypersensitivity reaction that is life-threatening and rarely seen in childhood. A five-year-old girl was admitted with complaints of extensive rash and fever a month after the onset of treatment with carbamazepine for a diagnosis of epilepsy. Physical examination revealed fever, an extensive maculopapular rash, submandibular lymphadenopathy and hepatomegaly. The patient was diagnosed with the DRESS syndrome as a result of the clinical and laboratory findings (presence of atypical lymphocytes, eosinophilia and hypertransaminasemia) and treated supportively together with systemic corticosteroids and intravenous immunoglobulin (IVIG). The DRESS syndrome should be considered in the differential diagnosis in children with a history of drug use presenting with fever, skin rash, lymphadenopathy and internal organ involvement. The basic principles in the treatment include early diagnosis, termination of suspected drugs and supportive therapy. Systemic corticosteroids and IVIG therapy are recommended, especially in severe cases

Anahtar Kelimeler

• Anticonvulsants, Child, DRESS syndrome, Hypersensitivity, Carbamazepine

Kaynakça

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3. Kardaun SH, Sidoroff A, Valeyrie-Allanore L, Halevy S, Davidovici BB, Mockenhaupt M, et al. Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: Does a DRESS syndrome really exist? Br J Dermatol 2007;156:609-11.
4. Knowles SR, Dewhurst N, Shear NH. Anticonvulsant hypersensiti- vity syndrome: An update. Expert Opin Drug Saf 2012;11:767-78.
5. Bocquet H, Bagot M, Roujeau JC. Drug-induced pseudolymphoma and drug hypersensitivity syndrome (Drug Rash With Eosinophilia and Systemic Symptoms: DRESS). Semin Cutan Med Surg 1996;15:250-7.