Dergiler / Turkish Journal of Hematology / 2016 / Cilt: 33 - Sayı: 2

Febril Nötropeni, Oral Kavitede Lezyonlar ve Deri Döküntüsü Olan Çocuklarda Stevens- Johnson Sendromu/Toksik Epidermal Nekrolizis Akılda Tutulmalıdır

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Should Be Kept in Mind in Children with Febrile Neutropenia, Oral Cavity Lesions, and Skin Rash

Sayfa
94–95
Yayın Tarihi
2022-11-05
DOI
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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

Cyclosporine, plasmapheresis, and tumor necrosisfactor-alpha inhibitors have been also reported among othertreatment options [1,2,3,4,5].

📌 3 Maddede Bu Makale

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Amaç & Hipotez:A 14-year-old boy was diagnosed with acute lymphoblasticleukemia.
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Temel Bulgular & Veri:Onfollow-up, a painful papular rash had appeared and oral mucositishad become worse (Figure 1).
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Sonuç & Katkı:Cyclosporine, plasmapheresis, and tumor necrosisfactor-alpha inhibitors have been also reported among othertreatment options [1,2,3,4,5].
🔬Tür:Literatür & Derleme Çalışması
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Özet

A 14-year-old boy was diagnosed with acute lymphoblasticleukemia. Febrile neutropenia developed during induction.Imipenem and teicoplanin were started because of severemucositis. Viral tests and bacterial cultures were unrevealing. Onfollow-up, a painful papular rash had appeared and oral mucositishad become worse (Figure 1). Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) was suspected. Intravenousimmunoglobulin (IVIG) at 1 g/kg/day and methylprednisoloneat 1 mg/kg/day were started. The lesions regressed in 1 week(Figure 2). Skin biopsy was consistent with SJS/TEN. Informedconsent was obtained.SJS and TEN are rare diseases characterized by fever and mucosaland cutaneous lesions [1]. It is defined as SJS when epidermalinvolvement affects less than 10% of the body surface area, asSJS/TEN overlap when the skin detachment ranges from 10%to 30%, and as TEN when it involves more than 30% [1,2]. Itmay occur after taking a new medication or may rarely have aninfectious origin. Our patient had no predisposing conditionsother than taking chemotherapeutic drugs and antibiotics. Themortality rate is high in SJS/TEN [1,3]. Rapid withdrawal ofthe probable causative drug(s) is important. Use of IVIGs andcorticosteroids is reported as the most commonly used therapyin childhood [1,4]. Systematic review of adult treatments for SJSand TEN did not show any benefit of these agents on mortalityrates [3]. Cyclosporine, plasmapheresis, and tumor necrosisfactor-alpha inhibitors have been also reported among othertreatment options [1,2,3,4,5].

Abstract

A 14-year-old boy was diagnosed with acute lymphoblasticleukemia. Febrile neutropenia developed during induction.Imipenem and teicoplanin were started because of severemucositis. Viral tests and bacterial cultures were unrevealing. Onfollow-up, a painful papular rash had appeared and oral mucositishad become worse (Figure 1). Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) was suspected. Intravenousimmunoglobulin (IVIG) at 1 g/kg/day and methylprednisoloneat 1 mg/kg/day were started. The lesions regressed in 1 week(Figure 2). Skin biopsy was consistent with SJS/TEN. Informedconsent was obtained.SJS and TEN are rare diseases characterized by fever and mucosaland cutaneous lesions [1]. It is defined as SJS when epidermalinvolvement affects less than 10% of the body surface area, asSJS/TEN overlap when the skin detachment ranges from 10%to 30%, and as TEN when it involves more than 30% [1,2]. Itmay occur after taking a new medication or may rarely have aninfectious origin. Our patient had no predisposing conditionsother than taking chemotherapeutic drugs and antibiotics. Themortality rate is high in SJS/TEN [1,3]. Rapid withdrawal ofthe probable causative drug(s) is important. Use of IVIGs andcorticosteroids is reported as the most commonly used therapyin childhood [1,4]. Systematic review of adult treatments for SJSand TEN did not show any benefit of these agents on mortalityrates [3]. Cyclosporine, plasmapheresis, and tumor necrosisfactor-alpha inhibitors have been also reported among othertreatment options [1,2,3,4,5].

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Bu Makaleye Atıf Yap

ATASEVEN, E., ÖREN, H., BENGOA, Ş. Y. (2022). Febril Nötropeni, Oral Kavitede Lezyonlar ve Deri Döküntüsü Olan Çocuklarda Stevens- Johnson Sendromu/Toksik Epidermal Nekrolizis Akılda Tutulmalıdır. Turkish Journal of Hematology, Cilt: 33 - Sayı: 2, 94–95. https://ajindex.com/articles/606829

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Kaynakça (5)

  1. Ferrandiz-Pulido C, Garcia-Patos V. A review of causes of Stevens-Johnson syndrome and toxic epidermal necrolysis in children. Arch Dis Child 2013;98:998-1003
  2. Mockenhaupt M. The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol 2011;7:803-813
  3. Roujeau J, Bastuji-Garin S. Systematic review of treatments for StevensJohnson syndrome and toxic epidermal necrolysis using the SCORTEN score as a tool for evaluating mortality. Ther Adv Drug Saf 2011;2:87-94
  4. Spies M, Sanford AP, Aili Low JF, Wolf SE, Herndon DN. Treatment of extensive toxic epidermal necrolysis in children. Pediatrics 2001;108:1162- 1168
  5. Scott-Lang V, Tidman M, McKay D. Toxic epidermal necrolysis in a child successfully treated with infliximab. Pediatr Dermatol 2014;31:532-534.