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
- Sayfa
- 94–95
- DOI
- —
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].