a 15-year - old female developed fever , stinging eyes and headache , followed one day later by sudden appearance of flaccid blisters on the face with subsequent dissemination to more than 70% of the whole integument and development of mucosal erosions .
thirty days before , she had been operated on the nose and medicated with cefuroxime ( for 8 days ) , paracetamol ( for approx .
10 days ) and metamizol ( for approx . 10 days ) , the latter of which was later identified by lymphocyte transformation test as the most likely causative compound .
four days before admission , she had taken acetylsalicylic acid and paracetamol for flu - like symptoms including conjunctival irritation and rhinitis . upon physical evaluation ,
disseminated flaccid blisters filled with serous liquid were present on 70% of her skin surface .
her eye- , mouth- , pharyngeal- and genital mucosae were also affected by erosive lesions .
she received intravenous immunoglobulins ( ivigs ) , in total 3 g / kg body weight over three consecutive days , hydration and nutritional support as well as careful wound care .
topical therapy included silver - coated dressings on erosions , and after reepithelialization , skin washes with water and wax emulsion were performed , followed by the application of hydrocortisone 0.5% in hydrophilic unguent . from day 1 after onset of ivig therapy onwards , no new development of blisters was observed . during the clinical course , desquamation of dusky areas of skin leaving an intact epidermis below was observed .
we noted frequent spontaneous bleeding upon changes of the silver - coated dressings during reepithelialization period in the second week .
the remaining detached skin fragments that overlied the progressively regrowing epidermis as of day 7 were progressively shed between day 7 and day 28 .
the patient was discharged from the intensive care unit ( icu ) at day 28 after admission , with almost complete skin reepithelialization . at the last follow - up control 6 months later , the patient showed full recovery , except for residual alopecia and dry eyes .
to date , no specific treatment with a high evidence level of efficacy has been reported for ten . the standard of care consists of best supportive therapy in an icu setting , and includes hydroelectrolytic and nutritional support as well as regular wound care and the appropriate detection and treatment of infectious complications . whereas best supportive care is an accepted standard of care for ten patients , none of the specific treatment strategies described to date , including ivig ( adequately dosed at 3 g / kg body weight
; there is an apparent dose dependence , as the reduction in mortality appears to be greatest for doses of 3 - 4 g / kg ) , corticosteroids , and infliximab , has shown solid evidence in support of a survival advantage in ten patients . to date , only one prospective , randomized - controlled clinical trial has been reported in ten , demonstrating no therapeutic benefit for thalidomide in the treatment of ten .
ivig is a safe therapeutic modality that has been shown in several case series to potentially provide a benefit in ten .
controversy as to the exact efficacy does , however , exist . in the recently established guideline for the use of high - dose immunoglobulins in dermatology , early administration of high - dose immunoglobulins ( 3 g / kg )
indeed , several published studies reporting the use of ivig for the treatment of ten have shown this therapy to potentially provide a survival benefit .
the studies published to date are unfortunately heterogeneous , and often of limited size , retrospective nature , and noncontrolled . on the other hand
, ten is a rare disease , and randomized prospective controlled studies analyzing the effect of ivig are very difficult to perform , and do not exist to date .
eight of the 11 studies published to date reporting the use of ivig in ten suggest a positive effect of ivig on mortality if compared to the mortality predicted by scorten where applied , or the 25 - 35% average mortality rate for ten reported in the literature ( table 1 ) .
it must be kept in mind , however , that as for many other therapies , the total dose ( in g / kg body weight ) of ivig administered may have an effect on the therapeutic outcome . indeed , a recent publication by trent et al . , analyzing in detail the studies published to date , showed that a dose - response relationship likely exists : per 1-g / kg increase in ivig dose , there was an observed 4.2-fold increase in patient survival .
moreover , no mortality was observed amongst the 30 patients that had received 3 g / kg ivig or more .
taking into account the lack of a proven specific therapy , the severity of ten , the safety of ivig , and the data to date concerning the efficacy of ivig in ten , we are of the opinion that in the absence of proven superior therapies , ivig therapy should be considered in patients with ten .
 toxic epidermal necrolysis is a rare but clinically well - described dermatological pathology . 
 however , clinical pictures of this disorder in text books do not reflect its dynamic evolution . 
 usually , the desquamative post - bullous stage is represented , neglecting the initial bullous stage as well as the skin healing . 
 with one clinical case , we provide a day - after - day illustration of the evolution of a patient suffering from toxic epidermal necrolysis . during one month , a skin area of a limb was regularly photo - documented . 
Case Report
Current Therapeutic Modalities for TEN