ABSTRACT
BACKGROUND: Toxic epidermal necrosis (TEN) is a severe and life-threatening syndrome manifesting as extensive necrosis of the epidermis and mucous membranes accompanied by systemic symptoms. The causative factor is exposure to drugs, such as sulphonamides, antiepileptic preparations, non-steroidal anti-inflammatory drugs and paracetamol. The mechanism leading to the development of lesions is unknown and there is no uniform strategy of management. CASE REPORT: A 4-year-old boy was admitted with late-stage TEN; he was additionally affected by partial atrophy of the corpus callosum, mental retardation and drug-resistant epilepsy. Three weeks before the first symptoms developed, antiepileptic treatment was widened with lamotrigine, which seems to be the causative factor of TEN. Since general and topical pharmacological therapy failed, plasmapheresis was applied and already after 2 cycles, the progression of lesions was inhibited and circulation was gradually stabilized. CONCLUSIONS: The use of plasmapheresis in a child with TEN can result in substantial improvement of the general and local condition. The case presented clearly indicates that standards of management of children with suspected or developed TEN should be urgently instituted.
Subject(s)
Anticonvulsants/adverse effects , Plasmapheresis/methods , Stevens-Johnson Syndrome/therapy , Triazines/adverse effects , Anticonvulsants/therapeutic use , Child, Preschool , Disease Progression , Drug Resistant Epilepsy/drug therapy , Humans , Lamotrigine , Male , Stevens-Johnson Syndrome/etiology , Stevens-Johnson Syndrome/physiopathology , Triazines/therapeutic useABSTRACT
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, are rare, life- -threatening diseases that are characterised by extensive epidermal detachment, erosion of mucous membranes and severe systemic symptoms. In the majority of cases, the development of symptoms can be attributed to the use of drugs; therefore, the disease pathology is thought to be caused by a severe adverse reaction to drugs. The high mortality rate results primarily from the development of complications in the form of systemic infections and multiple organ failure. TEN and SJS affect all age groups, including newborns, infants and older children. The rarity of these syndromes has not permitted large, randomised studies, which has resulted in numerous difficulties in their diagnosis and management. Because the pathogenesis has not yet been established, the management and systemic treatment of these syndromes have not been standardised. The efficacy of the treatment options suggested has not been confirmed by clinical studies involving suitably large groups of patients, especially children.