toxic epidermal necrolysis ( ten ) , also known as lyell 's syndrome , is a widespread life - threatening mucocutaneous disease where there is extensive detachment of the skin and mucous membrane characterized by full thickness necrosis of the epidermis .
patients initially develop pain , tenderness or a burning sensation in the skin associated with fever and malaise , which generally begin abruptly . over the next 1 to 3 days , ill - defined erythematous macules or a diffuse erythema develops over the trunk and extremities .
as the red areas enlarge , central dusky necrotic sites develop with subsequent bullae formation . as the disease progresses ,
a positive nikolsky sign ( peeling - off of the epidermis in large sheets caused by a sliding touch ) is an important diagnostic clue and precedes the onset of a life - threatening event .
steven - johnson syndrome ( sjs ) involves less than 10% epidermal detachment and ten involves more than 30% of epidermal detachment . between 10%29% epidermal detachment
the drugs blamed includes sulphonamides , aminopenicillins , quinolones , cephalosporins , carbamazepine , phenobarbital , phenytoyn , nsaids , and allopurinol .
here we describe a case of toxic epidermal necrolysis in an adult male patient receiving carbamazepine for 3 weeks for complex partial seizures .
a 38-year - old asian indian male patient presented with a rapidly progressive generalized eruption with fever .
patient was diagnosed as having secondarily generalized complex partial seizures 3 weeks prior to the current illness and was prescribed carbamazepine at the dose of 100 mg twice daily with slow up - titration of the dose to 300 mg twice daily . on the18th day of carbamazepine , patient developed blisters which first appeared on the trunk , chest , and arms .
later patient developed fluid filled lesions all over the body , eyes , mouth and genitalea .
physical examination showed hypotension ( systolic blood pressure 90mmhg ) and tachypnoea ( respiratory rate 50/minute ) .
clinical examination of the skin revealed a generalized peeling of the skin with crusting seen almost all over the body including scalp and genitalea [ figure 1 ] .
the erythematous rash was covering almost all over the body with epidermal detachment of 70% body surface area .
there was loss of eye lashes , congestion of conjunctiva with mucopurulent discharge and exposure keratitis .
his total white blood cell count was 4.59  10/l ( normal 410  10/l ) .
liver function tests showed elevated aspartate aminotransferase ( ast ) 170 ( normal 1045 ) u / l and elevated alanine aminotransferase ( alt ) 87 ( normal648 )
7.9mmol / l ) , which was corrected appropriately . wound swabs of the lesions grew staphylococcus aureus and psuedomonas aeruginosa .
generalized peeling of the skin in toxic epidermal necrolysis the clinical impression was ten induced by carbamazepine .
the erosions were smeared with fusidic acid and betamethasone combination and covered with sterile paraffin gauge .
the patient was made to lie down on sterile banana leaf to prevent sticking of the skin to cotton bed .
eye lesions were treated with topical antibiotic preparations ( ciprofloxacin , gentamicin , chloramphenicol , moxifloxacin+dexamethasone ) and ocular lubricant solution ( lacrigel ) .
supportive treatment given includes parenteral opioids ( fentanyl , pentazocine ) for pain management , intravenous fluids and intravenous albumin .
patient was started on levetiracetam for complex partial seizures on the fourth day of admission .
after one month , the progression of the skin lesions halted and general condition of the patient was improved . as a sequelae , on follow - up patient had developed hypertrophic scars in some areas .
our patient presented with generalized peeling of the skin with mucosal ulceration after initiating the therapy with carbamazepine .
several case reports have shown that carbamazepine is one of the culprits in inducing ten .
incubation period for development of ten after initiating carbamazepine was less than 3 weeks , which is in conformity with earlier reports .
our patient had a low white cell count , which is more commonly found in ten .
specific epidemiological studies have not been published on the incidence of ten in developing countries like india .
devi et al reported that the most common [ 53% ] group of drugs implicated as the cause of sjs / ten was anticonvulsants and majority of these cases [ 81% ] were due to carbamazepine .
it is possible that frequency of ten in countries such as india could be much higher considering the fact that the main etiologic agent of ten , i.e. drugs such as antibiotics , anticonvulsants , and nonsteroidal anti - inflammatory drugs ( nsaids ) are easily available in these countries without any prescription .
cytotoxic lymphocyte - mediated immune reaction ( ctl ) aimed at the destruction of the keratinocyte expressing foreign antigens as the primary causes for development of ten .
tumor necrosis factor - a ( tnf - a ) and interleukin-6 ( il-6 ) have also been involved in the pathogensis of ten as increased amount of these cytokines are found in the blister fluids in ten patients .
these inflammatory cytokines may play their damaging roles by recruiting the cytotoxic t cells to the epidermis .
they may also cause damages directly as cytokines such as tnf - a are known to cause increased apoptosis .
drug metabolites such as hydroxylamines and arene oxides derived from sulfonamides and aromatic anticonvulsants , respectively , bind to cell constituents if they are not rapidly detoxified by epoxide hydrolase .
these metabolites act as haptens and render the keratinocytes antigenic by binding to them . a defect in the detoxification system may be the cause of drug eruption .
a strong association has been reported between human leucocyte antigen ( hla)-b1502 and carbamazepine - induced sjs in han chinese patients .
european studies suggested that hla - b1502 is not a universal marker but is ethnicity specific for asians .
biopsy of the specimen shows necrosis of basal layer cells without massive inflammatory infiltration of the dermis ; in specimens with mature lesions it is possible to observe necrosis of keratinocytes involving all the epidermis with diffuse detachment of the epidermis at the level of the basal membrane .
other possible medical therapies for the treatment of ten that are reported in the literature include the use of plasmapheresis , intravenous immunoglobulins , and cyclosporine .
complete recovery of our patient was probably due to early and effective control of inflammation by treatment with systemic steroids and appropriate topical care of the eroded areas .
as reported in the earlier literature , most of the patients with ten were treated with systemic steroids along with antibiotics and supportive measures .
apart from control of seizures , carbamazepine is being increasingly prescribed for control of pain in neuralgias and diabetic neuropathy .
awareness about the drugs implicated in life threatening drug reactions will help physicians in preventing them by judicious use of the drugs .
 toxic epidermal necrolysis ( ten ) , also known as lyell 's syndrome , is a widespread life - threatening mucocutaneous disease where there is extensive detachment of the skin and mucous membrane . 
 many factors involved in the etiology of ten including adverse drug reactions . 
 here we are reporting a case of toxic epidermal necrolysis in an adult male patient after receiving carbamazepine in a 38 year old male . on the 18th day of carbamazepine 
 , patient developed blisters which first appeared on the trunk , chest and arms . 
 the erythematous rash was covering almost all over the body with epidermal detachment of 70% body surface area . 
 there was loss of eye lashes , congestion of conjunctiva with mucopurulent discharge and exposure keratitis . 
 the clinical impression was ten induced by carbamazepine . 
 carbamazepine was stopped immediately . 
 he was treated with high dose intravenous betamethasone and systemic and topical antibiotics . 
 after one month , the progression of the skin lesions halted and he was discharged . 
Introduction
Case Report
Discussion