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drug rash with eosinophilia and systemic symptoms ( dress ) syndrome is a rare and life - threatening delayed drug hypersensitivity reaction characterized by skin eruption , fever , lymphadenopathies , and visceral involvement .
lamotrigine [ 6-(2,3-dichlorophenyl)-1,2,4-triazine- 3,5-diamine ] is an antiepileptic drug , effective for a broad range of seizures in adults and children , which is structurally and pharmacologically unrelated to other antiepileptic medications .
several reports of lamotrigine - induced dress syndrome have been reported in the literature.[35 ] here we report a case of dress syndrome induced by lamotrigine , which was detected early and completely responded to the treatment / thereby reducing the morbidity in the patient .
a 12 year old boy , with a known case of seizure disorder presented to the casualty with generalized maculopapular rash , fever and facial puffiness .
the patient had been operated for gastric perforation when he was 3 days old following which the seizure episodes started .
the patient was taking 1 tablet of 25 mg daily for the first 15 days ; later the dose was doubled .
on the 18 day , patient presented with generalized maculopapular rash , itching and fever and on 20 day he developed colicky abdominal pain and vomiting followed by facial puffiness . on examination , he was alert and oriented , with a temperature of 100f and pulse was 114/ minutes .
there was significant angioedema of his face with edematous lips and swollen ears , and an extensive maculo - papular rash on his entire trunk [ figure 1 ] , buttocks , extremities and face .
he experienced no joint pain or neurological symptoms , and no abdominal or respiratory complaints .
he had 3 siblings : one of them also had history of epileptic disorders for which she was on treatment .
maculo - papular rashes on the trunk investigations revealed leukocytosis ( 15,960cells / cmm ) with eosinophilia ( 19% ) aec=2600/cmm .
hemoglobin was 10.8gm% , platelet count 2.53lakhs / cmm ; esr and pcv were normal .
liver enzymes were raised with sgot=905u / l , sgpt=495u / l , alp=855u / l and ggt= 155u / l .
drug hypersensitivity syndrome ( dhs ) , recently being also referred to as dress ( drug reaction with eosinophilia and systemic symptoms ) or didmohs ( drug - induced delayed multi - organ hypersensitivity syndrome ) , is a distinct type of adverse drug reaction .
the skin , liver and hematologic system are most commonly involved , with cutaneous changes being the most apparent .
the presence of the 2 criteria , as given by bocquet et al . , has high sensitivity ( > 95% ) but weak specificity ( < 80% ) .
there was eosinophilia , lymphadenopathy and liver enzymes were raised , which satisfied the criteria , and established a diagnosis of dress .
prompt resolution of the symptoms after the withdrawal of the lamotrigine , further supported the diagnosis .
lamotrigine was the probable cause of dress syndrome in the present case , as assessed by the naranjo causality criteria .
diagnostic criteria for drug hypersensitivity syndrome the liver is the most commonly involved organ in dress .
the degree of hepatitis is related to the interval between the onset of the syndrome and the discontinuation of the anticonvulsant .
associated infection by human herpes virus 6 may also play a role in its development .
hypersensitivity reactions to aromatic antiepileptic drugs appear to have an immune etiology , much like the lamotrigine - induced reaction : bio activation , detoxification , covalent adduct formation , processing and presentation of antigen to the immune system , and consequent formation of antibody and t - cell immune effectors .
these are usually detoxified by the enzyme epoxide hydrolase , which may be lacking or mutated in persons that develop the syndrome .
it is chiefly metabolized by hepatic glucuronidation ; the resulting metabolite has no pharmacologic activity and is excreted in urine .
our patient was comfortable when he was receiving single dose of lamotrigine , but he developed dress , 3 days after the dose was doubled .
this may be explained by the fact that , there may be a threshold concentration of lamotrigine above which the body 's ability to metabolize the drug may be overwhelmed , leading to a hypersensitivity reaction . in pediatric age group who are on sodium valproate
the incidence of dhs ( approximately 1 in 1,000 to 1 in 10,000 exposures ) is probably underestimated .
though no standard treatment has been proposed , intravenous corticosteroids may be effective . in severe cases , pulse therapy with high dose of intravenous methyl prednisolone , plasmapharesis and human intravenous immunoglobin
lamotrigine treatment has been associated with multiorgan failure , dress syndrome , acute hepatic failure , and disseminated intravascular coagulation .
we suggest that these potentially fatal side effects should be considered in any patient when clinical deterioration follows treatment with this drug .
early recognition and withdrawal of the suspected agent may avoid irreversible damage to the liver and will be life saving .
this case reiterates the necessity of early recognition of this syndrome , especially when lamotrigine is given concurrently with sodium valproate .
it helps in the rapid resolution of dress , thereby mitigating the morbidity and mortality in the patient .
drug rash with eosinophilia and systemic symptoms ( dress ) syndrome is a rare and life - threatening delayed drug hypersensitivity reaction characterized by skin eruption , fever , lymphadenopathies , and visceral involvement . here , we are presenting a 12 year old boy , who developed rare but life threatening dress syndrome due to lamotrigine . 
 early detection and treatment led to his rapid recovery . 
 this case is presented to highlight the importance of early detection of rare fatal syndrome .