Coexistence of Stevens-Johnson syndrome and hemophagocytic syndrome

Toxic epidermal necrolysis and hemophagocytic lymphohistiocytosis: a case report and literature review

“…Our patient's favorable outcome is consistent with other pediatric case reports [11,12,17,18]. One patient relapsed [19], but was effectively managed, demonstrating the need for close surveillance. One fatality did occur, however [12], with a patient experiencing considerable comorbidities including chronic renal failure requiring hemodialysis.…”

Epstein-Barr infection causing toxic epidermal necrolysis, hemophagocytic lymphohistiocytosis and cerebritis in a pediatric patient

“…CNS involvement is not included in the diagnostic criteria; however this is a common manifestation in HLH with an incidence of up to 70% and can occur either at presentation or throughout the course of disease progression. [20].…”

Stevens-Johnson syndrome complicated by fatal hemophagocytic lymphohistiocytosis

“…To date, there have been 8 published cases of SJS/TEN complicated by HLH (Table II). 3, 4, 5, 6, 7, 8, 9, 10 The pathogenesis of this possible association is unknown. In SJS/TEN, analysis of blister fluid shows interferon gamma as the predominant cytokine.…”

“…Both processes involve increased circulating CD8 + T cells; in HLH, CD8 + T cells are unable to be downregulated, whereas in SJS/TEN they are inappropriately activated, leading to keratinocyte apoptosis 3 Table IIDocumented cases of SJS/TEN complicated by HLHPatientSuspected triggerPertinent historyTreatment of HLHOutcomePediatric 7-month-old boy 4 Ceftriaxone sodiumBronchitisCyclosporine A, methylprednisoloneDischarged healthy on day 25 17-month-old boy 3 Dicloxacillin, cephalexin, ibuprofenTest results for laryngotracheitis, HHV-6, parainfluenza, and rhinovirus positiveEtoposide, dexamethasoneFully recovered after 2 months 2-year-old girl 5 EBV infectionReceived IVIG, aspirin, flucloxacillin, gentamicin, and clindamycinSteroids, methotrexate, etoposide, rituximabAfter bone marrow transplant, healthy 8 months later 4-year-old boy 6 Cephalosporin, ibuprofenURIHigh-dose IVIG, methylprednisoloneDischarged healthy 12-year-old boy 7 Meropenem, vancomycin, sodium valproateOn dialysis, contracted MRSAGM-CSF, blood transfusionsDied of sepsis and renal failure 16-year-old girl 8 EBV infectionEBV-infected CD8 + T lymphocytes on skin biopsyEtoposide and dexamethasoneImproved with mild residual erythema and desquamation of skinAdult 34-year-old woman 9 AntidepressantsParvovirus-B19 infectionHigh-dose γ-globulin and plasmapheresisDeveloped MRSA infection and DIC and died of brain hemorrhage 76-year-old woman 10 EtodolacN/AN/ADied due to sepsis and hepatic failure DIC , Disseminated intravascular coagulation; EBV , Epstein-Barr virus; HHV - 6 , human herpesvirus 6; HLH , hemophagocytic lymphohistiocytosis; IVIG , intravenous immunoglobulin; MRSA , methicillin-resistant Staphylococcus aureus ; N / A , not applicable; SJS / TEN , Stevens-Johnson syndrome/toxic epidermal necrolysis; URI , upper respiratory infection.…”