A comparative study of human immunoglobulin versus recombinant human tumor necrosis factor-α receptor Ⅱ:IgG Fc fusion protein in the treatment of toxic epidermal necrolysis / 中华皮肤科杂志

Objective:To evaluate and compare efficacy of intravenous immunoglobulin (IVIG) versus recombinant human tumor necrosis factor-α receptor Ⅱ:IgG Fc fusion protein (rhTNFR:Fc) in the treatment of toxic epidermal necrolysis (TEN) .Methods:Clinical data were collected from patients with TEN treated with IVIG or rhTNFR:Fc in Wuhan No.1 Hospital from 2013 to 2019. There were 11 patients in the IVIG group, including 3 males and 8 females, aged 25-72 years, and the median TEN-specific severity-of-illness score (SCORTEN) was 3 points; there were 10 patients in the rhTNFR:Fc group, including 5 males and 5 females, aged 32-84 years, and the median SCORTEN was 2 points. These patients all showed no response to the 5-day treatment with prednisolone acetate at a dose of 0.6-1.0 mg·kg -1·d -1, and then received IVIG at a dose of 400 mg·kg -1·d -1 for 5 consecutive days, or subcutaneous injection of rhTNFR:Fc at a dose of 25 mg every other day for 4-6 sessions. Changes in skin lesions and adverse events were recorded in the 2 groups. Statistical analysis was carried out by using Mann-Whitney U test. Results:Compared with the rhTNFR:Fc group, the IVIG group showed a significant decrease in the time to onset of reduction of skin lesion exudate (1.73 ± 1.19 days vs. 3.00 ± 1.56 days, P < 0.05) , time to onset of pain relief in the lesion area (1.64 ± 1.28 days vs. 3.70 ± 1.63 days, P < 0.05) , time to lightening of color of the lesion base (2.45 ± 1.12 days vs. 3.90 ± 1.59 days, P < 0.05) , time to onset of new epidermis growth (3.09 ± 1.13 days vs. 5.20 ± 1.22 days, P < 0.05) , and in the time to onset of lesion drying at the intertriginous sites (4.82 ± 2.22 days vs. 7.90 ± 3.14 days, P < 0.05) . However, there was no significant difference in the length of hospital stay between the IVIG group (17.70 ± 8.33 days) and rhTNFR:Fc group (16.70 ± 4.71 days, P > 0.05) . No adverse reactions were observed during the treatment, and no recurrence or complications were found in the 21 patients during the follow-up of 6 months. Conclusion:IVIG and rhTNFR:Fc are both effective in the treatment of TEN, but IVIG is superior to rhTNFR:Fc in terms of the time to onset of pain relief, skin lesion exudate reduction and epidermal growth.

Comparison of the performance of SCORTEN and ABCD-10 in evaluating the prognosis of Stevens-Johnson syndrome/toxic epidermal necrolysis / 中华皮肤科杂志

Objective:To compare the performance of the severity-of-illness score for toxic epidermal necrolysis (SCORTEN) and ABCD-10 (age, bicarbonate, cancer, dialysis, 10% body surface area) scoring systems in predicting death in patients with Stevens-Johnson syndrome (SJS) /toxic epidermal necrolysis (TEN) .Methods:Clinical data were collected from 85 patients with SJS/TEN who were hospitalized in Sichuan Provincial People′s Hospital from January 2010 to April 2021, and retrospectively analyzed. The predicted mortality and actual mortality were compared at each score level of SCORTEN and ABCD-10. The receiver operating characteristic (ROC) curve and Hosmer-Lemeshow goodness-of-fit test were used to evaluate the predictive power and calibration of SCORTEN and ABCD-10 on mortality.Results:Among the 85 patients, 37 were males and 48 were females, and their ages were 52.36 ± 19.31 years (range, 14 - 88 years) . There were 61 cases of SJS, 6 of SJS/TEN overlap, and 18 of TEN. Ten patients died in hospital and the fatality rate was 11.76%. Among the SCORTEN and ABCD-10 components, age > 40 years or ≥ 50 years, epidermal exfoliation > 10% body surface area on the 1st day after admission, heart rate > 120 beats per minute, serum urea nitrogen level > 10 mmol/L and serum bicarbonate level < 20 mmol/L were significantly correlated with death ( χ2 = 4.46, 6.18, 25.50, 15.13, 7.59, 8.38, respectively, all P < 0.05) , while malignancies, serum glucose level > 14 mmol/L, and pre-hospital dialysis were not significantly correlated with death ( χ2 = 0.35, 0.10, 1.38, respectively, all P > 0.05) . There were no significant differences between the predicted mortality and actual mortality at every score level of SCORTEN and ABCD-10 (all P > 0.05) . The ROC curve showed that both SCORTEN and ABCD-10 had good predictive power for death (areas under the curve: 0.874 and 0.867, 95% CI: 0.758 - 0.990, 0.773 - 0.962, respectively) , but the model goodness-of-fit of SCORTEN was superior to that of ABCD-10 ( P = 0.944, 0.048, respectively) . Conclusion:Both SCORTEN and ABCD-10 scoring systems could accurately predict mortality of SJS/TEN patients at early stage, but SCORTEN showed more favourable predictive power and calibration.

Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients

Abstract: Background: Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term 'Epidermal Necrolysis' has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome and toxic epidermal necrolysis at opposite ends, which differ by the extent of body surface area with epidermal detachment. SCORTEN is a mortality prognosis score for 'Epidermal Necrolysis' cases that still needed validation in acquired immunodeficiency syndrome. Objective: To evaluate the SCORTEN performance in acquired immunodeficiency syndrome, and the differences in outcomes between acquired immunodeficiency syndrome and non- acquired immunodeficiency syndrome cohorts. Methods: Retrospective cohort study of AIDS and non-AIDS 'Epidermal Necrolysis' cases admitted to a Brazilian reference center from 1990-2014. Results: Five deaths (16.7%) occurred as a consequence of EN in 30 AIDS patients, and seven (17.9%) in 39 non-AIDS patients, relative risk (RR) .92 (p=1.0). SCORTEN showed great performance, with an Area Under the Receiver Operating Curve (AUC) (ROC) of 0.90 with a 95% confidence interval ranging from .81 to .99. The performance of SCORTEN was better among non- AIDS patients than AIDS patients: AUC non- acquired immunodeficiency syndrome =0.99 (CI 05% 0.96-1.00), AUC acquired immunodeficiency syndrome = 0.74 (CI 95% 0.53-0.95), p=.02. Study Limitations: Heterogeneity of cases, wide variation of systemic corticosteroid doses when used. Conclusion: SCORTEN is valid for the Brazilian population, including among those patients with acquired immunodeficiency syndrome, and, as such, its use is recommended for aiding treatment choice in this subgroup of patients.

Effect of interferon-γ combined with tumor necrosis factor-related apoptosis-inducing ligand on programmed necrosis of HaCaT cells and its mechanisms / 中华皮肤科杂志

Objective To evaluate the inductive effect of interferon-γ(IFN-γ) combined with tumor necrosis factor (TNF)-related apoptosis-inducing ligand (TRAIL) on programmed necrosis of the human immortalized keratinocyte cell line HaCaT,and to explore its mechanisms.Methods In vitro cultured HaCaT cells were divided into several groups:negative control group receiving no treatment,IFN-γ group treated with 50 μg/L IFN-γ,TRAIL group treated with 4 μg/L TRAIL,and cytokine combination group treated with 50 μg/L IFN-γ and 4 μg/L TRAIL or zVAD combination group pretreated with 40 μmo/L zVAD for 1 hour followed by the treatment with 50 μg/L IFN-γand 4 μg/L TRAIL.After 48-hour treatment,the morphology of HaCaT cells were observed under a light microscope,methyl-thiazolyl-tetrazolium assay was performed to evaluate the inhibitory effect of the treatment on the proliferation of HaCaT cells,and double staining flow cytometry to detect the necrosis of HaCaT cells.Meanwhile,real-time fluorescence-based quantitative PCR (qPCR) was conducted to determine the mRNA expression of receptor interaction protein kinase 3 (RIP3) and mixed lineage kinase domain-like protein (MLKL),Western blot analysis to determine the expression of RIP1,RIP3,MLKL proteins and their phosphorylated forms (pRIP1,pRIP3,pMLKL),immunofluorescent staining to observe the distribution of pRIP3 and pMLKL in HaCaT cells,and the level of reactive oxygen species (ROS) in HaCaT cells in the above groups was detected by the fluorescence probe DCFH-DA.Statistical analysis was carried out with SPSS 22 software by using one-way analysis of variance (ANOVA) for comparing indices among different groups,and least significant difference (LSD)-t test for multiple comparisons.Results After 48-hour treatment,HaCaT cells in the cytokine combination group and zVAD combination group showed necrosis-like morphologic features.Methyl-thiazolyl-tetrazoliumassay revealed significant differences in the survival rate of HaCaT cells among the IFN-γgroup,TRAIL group,cytokine combination group,zVAD combination group and negative control group (73.16％ ± 5.71％,81.46％ ± 4.68％,72.18％ ± 2.93％,69.67％ ± 3.24％ and 100％,respectively;F =24.34,P ＜ 0.001).The necrosis rate of HaCaT cells was notably higher in the cytokine combination group and zVAD combination group (9.86％ ± 1.31％,10.33％ ± 2.16％,respectively) than in the negative control group (5.26％ ± 0.91％,t =4.61,5.07,respectively,both P ＜ 0.05).qPCR revealed that the mRNA expression of RIP3 and MLKL significantly increased in the cytokine combination group and zVAD combination group compared with the negative control group (tRIP3 =0.99,1.84,tMLKL =1.51,2.17,respectively,all P ＜ 0.05).Western blot analysis suggested that the protein expression of RIP1,RIP3,MLKL,pRIP1,pRIP3 and pMLKL significantly increased in the cytokine combination group compared with the negative control group (all P ＜ 0.05),and the zVAD combination group showed significantly decreased caspase 8 expression and increased expression of the above proteins compared with the cytokine combination group.Fluorescence microscopy showed that enhanced green dot-like or clump-like fluorescent spots (representing pRIP3) could be observed in the cytoplasm,and red fluorescent spots (representing pMLKL) could be seen on the cell membrane in the cytokine combination group.The average fluorescence intensity of ROS was significantly higher in the cytokine combination group than in the negative control group (t =702.00,P ＜ 0.05).Conclusion IFN-γcombined with TRAIL can induce the programmed necrosis of HaCaT cells with increased level of ROS.

Efficacy of combined lymphoplasma exchange for the treatment of severe refractory immune-related skin diseases: a clinical observation / 中华皮肤科杂志

Objective To evaluate the clinical efficacy of lymphoplasma exchange (LPE) for the treatment of severe refractory immune-related skin diseases.Methods From May 2013 to October 2015,8 patients with toxic epidermal necrolysis,drug-induced hypersensitivity syndrome (DIHS),pemphigus vulgaris,pemphigoid or paraneoplastic pemphigus were enrolled from Department of Dermatology,Xiangya Hospital,Central South University,who showed no response to conventional therapy or presented with multiple organ dysfunction.After the treatment with LPE,the efficacy was evaluated,and adverse reactions were observed.Results After one session of LPE therapy,6 patients received marked improvement,and were cured at last.In 1 patient with pemphigus vulgaris who was resistant to the treatment with high doses of glucocorticoids and immunosuppressive agents,the rashes regressed during the treatment with LPE,but recurred after the end of treatment.One patient with bullous pemphigoid presented with eruptive blisters on the next day after the treatment with LPE,which were considered as allergic reactions to allogeneic plasma.There were no obvious differences in white blood cell count,lymphocyte count,neutrophil count and blood platelet count in the peripheral blood of 8 patients before and after the treatment with LPE.During the follow-up of 3-5 years,all of the patients were recovered without recurrence,except 1 patient with bullous pemphigoid who died of disseminated tuberculosis after 1 year.Conclusion LPE is effective for the treatment of severe immune-related skin diseases,but attention should be paid to potential transfusion reaction and allergic reactions.

Prognostic accuracy of the SCORTEN scoring system in patients with toxic epidermal necrolysis or Stevens-Johnson syndrome / 中华皮肤科杂志

Objective To evaluate the prognostic accuracy of the score of toxic epidermal necrolysis (SCORTEN) scoring system in patients with toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS).Methods Clinical data were collected from 39 patients with SJS/TEN hospitalized in Peking Union Medical College Hospital during April 1992 and March 2014,and retrospectively analyzed.Among the 39 patients,13 had died,and the other 26 patients,who were matched to the dead patients in a ratio of 2:1 for age,all had a definite diagnosis and were discharged with improved conditions.The SCORTEN scoring system was used to evaluate the 39 patients with SJS/TEN and calculate expected mortality.The expected mortality and actual mortality were compared between different groups stratified by age in the 39 patients.The receiver operating characteristic (ROC) curve was drawn to assess the prognostic accuracy of the SCORTEN scoring system.Results According to the SCORTEN scoring system,15 out of the 39 patients scored 1 point,14 scored 2 points,6 scored 3 points,and 4 scored 4 points.The total number of expected deaths was 6.808,while that of actual deaths was 13.There was no significant difference between the expected mortality and actual mortality in every SCORTEN score-based group.The area under curve (AUC) was 0.832 8,indicating a good predictive ability of the SCORTEN scoring system.Conclusion The SCORTEN scoring system can predict mortality in TEN/SJS patients at early stage.

Evaluation of efficacy of glucocorticoids and intravenous immunoglobulin for the treatment of toxic epidermal necrolysis / 中华皮肤科杂志

Objective To evaluate the efficacy of systemic glucocorticoids and intravenous immunoglobulin (IVIG)for the treatment of toxic epidermal necrolysis (TEN). Methods Clinical data on TEN inpatients treated with systemic glucocorticoids alone or in combination with IVIG were collected from the Department of Dermatology, First Affiliated Hospital of Nanjing Medical University from January 2006 to December 2012. Therapeutic outcomes were evaluated in these patients. Statistical analysis was carried out by using a multiple linear regression analysis, binary logistic regression analysis and Cox regression analysis with the SPSS 16.0 software. Results A total of 48 inpatients with TEN were included in this study. Multiple linear regression analysis showed that the maximum daily dose of glucocorticoids for disease control was decreased gradually over years (β=-0.461, P=0.004). However, binary logistic regression analysis revealed no obvious changes in the frequency of administration of IVIG over years. Cox regression analysis showed that both hospitalization duration (RR=0.351, 95.0%CI:0.150-0.825)and the time required for the control of skin lesions (RR=0.492, 95.0%CI:0.245-0.986)decreased with the increase in the frequency of IVIG administration. In addition, with the increase in the maximum daily dose of glucocorticoids for disease control, the time required for the control of skin lesions was also shortened (RR=0.997, 95.0%CI:0.994 -1.000), while no obvious changes were observed in hospitalization duration. Conclusions IVIG shows superiority in controlling lesions, reducing complications and improving the prognosis of TEN. Compared with systemic glucocorticoids, IVIG shows better therapeutic efficacy and less adverse effects, and may be preferentially selected.

Methazolamide-induced toxic epidermal necrolysis in a patient with HLA-B5901 allele / 中华皮肤科杂志

A 56-year-old female patient of Han nationality presented with generalized erythema and vesicles for 6 days,as well as high fever for 2 days.Twenty days prior to hospitalization,the patient received surgical treatment combined with oral methazolamide and glucocorticoids for glaucoma.The patient had a history of allergy to sulfanilamides.On admission,the patient presented with generalized erythema,vesicles and occasional erosions with bilateral eyelid and oral involvement.Nikolsky's sign was positive.Wheezing sound was heard over the right lung.Genetic testing showed that HLA-B5901 allele was positive.The patient was diagnosed with methazolamide-induced toxic epidermal necrolysis (TEN) complicated by pneumonia,and managed with immunoglobulin (25 g/day,5 days),glucocorticoids (the largest dose equivalent to methylprednisolone 160 mg/day),fresh plasma,antibiotics,as well as other supporting and symptomatic treatments.The condition was controlled after 2 weeks,and the patient was cured and discharged from hospital after 25 days.The fact that the patient carried HLA-B5901 allele suggests that HLA-B5901 is strongly correlated with methazolamide-induced TEN or Stevens-Johnson syndrome in Chinese descendants or Han population,besides in Japanese and Korean descendants.

Diagnosis and treatment of severe acute pancreatitis complicated with toxic epidermal necrolysis / 中华消化外科杂志

Toxic epidermal necrolysis (TEN) is an acute skin inflammation,which belongs to heavy drug eruption.With the extensive and widespread use of antibiotics,nonsteroidal antipyretic analgesics and other pharmacotherapies in the treatment of critically ill patients,the trend of morbidity of TEN is gradually increased,especially in intensive care unit.In consideration of the high mortality rate of TEN and its great influence on the overall prognosis of patients with critical illness,we summarized the diagnosis and treatment experiences in the management of TEN based on retrospective analysis of one patient with severe acute pancreatitis and TEN.

Treatment of toxic epidermal necrolysis with intravenous immunoglobulin: a series of three cases / Tratamento da necrólise epidérmica tóxica com imunoglobulina endovenosa: uma série de três casos

Stevens-Johnson's syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatoses, that lead to keratinocyte apoptosis induced by interactions between Fas (cell death receptor) and soluble Fas-ligand, present in serum of Stevens-Johnson's syndrome / toxic epidermal necrolysis patients. Anti-Fas antibodies in intravenous immunoglobulin (IVIG) would block the apoptosis cascade. Three cases of toxic epidermal necrolysis occurred in one male and two female patients, after use of allopurinol, leprosy multidrug therapy concomitant with dipyrone, and diclofenac. The cases were treated with intravenous immunoglobulin 2-3 mg/kg and prednisone 20-50 mg/day. The interruption of new lesions outbreak and reepithelization were extremely fast after the use of intravenous immunoglobulin, without adverse effects. Controlled studies are needed to confirm the efficacy of intravenous immunoglobulin in Stevens-Johnson's syndrome / toxic epidermal necrolysis, but the results seem promising.

A Síndrome de Stevens-Johnson e a Necrólise Epidérmica Tóxica são dermatoses graves, que levam à apoptose dos queratinócitos induzida pela interação entre Fas (receptor de morte celular) e Fasligante solúvel, presente no soro de pacientes com Síndrome de Stevens-Johnson e Necrólise Epidérmica Tóxica. Anticorpos anti-Fas contidos na imunoglobulina endovenosa podem bloquear esta cascata apoptótica. Três casos de Necrólise Epidérmica Tóxica são descritos, ocorrendo após uso de alopurinol, diclofenaco e poliquimioterapia para hanseníase concomitante com dipirona. Os três casos foram tratados com imunoglobulina endovenosa 2-3 mg/kg, divididos em 4 ou 5 dias e prednisona 20-50 mg/dia. A interrupção no surgimento de novas lesões e a repitelização foram extremamente rápidas, sem ocorrência de efeitos adversos. Estudos controlados são necessários para confirmar a eficácia da imunoglobulina endovenosa na Síndrome de Stevens-Johnson e Necrólise Epidérmica Tóxica, porém, seus resultados parecem ser promissores.

Diagnósticos de enfermagem para o paciente com necrólise epidérmica tóxica: estudo de caso / Nursing diagnoses for the patient with toxic epidermal necrolysis: a case study / Diagnósticos de enfermería para el paciente con necrólisis epidérmica tóxica: estudio de caso

Trata-se de um estudo de caso clínico, retrospectivo, de um paciente acometido por necrólise epidérmica tóxica, internado em uma unidade de terapia intensiva de uma organização hospitalar pública, com o objetivo de apreender, a partir dos julgamentos clínicos das (os) enfermeiras (os), os diagnósticos de enfermagem. Foram evidenciados treze diagnósticos de enfermagem, assim como a necessidade de aprimoramento teórico destas profissionais sobre a Sistematização da Assistência de Enfermagem e o sentido de valor que esta prática pode acrescentar à profissão, na condução da assistência individualizada aos pacientes sob seus cuidados.

This is a retrospective case study of a patient affected by toxic epidermal necrolysis in the intensive care unit of a public hospital, with the goal to apprehend, starting from the clinical judgments of the nurses, theirs nursing diagnoses. Thirteen nursing diagnoses were evidenced and, also, it was evidenced the necessity of the theoretical improvement of those professionals about the Systematization of Nursing Care, and on the sense of value that this practice may add to nursing in the pursuit of individualized assistance to the patients under their care.

Es un estudio de caso clínico, retrospectivo, de un paciente afectado por necrólisis epidérmica tóxica, internado en una unidad de terapia intensiva de una organización hospitalaria pública, con el objetivo de aprehender, a partir de los juicios clínicos de las(los) enfermeras(os), los diagnósticos de enfermería. Fueran evidenciados trece diagnósticos de enfermería, así como la necesidad de actualización teórico-práctica de estas profesionales, sobre la Sistematización de la Asistencia de Enfermería y el sentido de valor que esta práctica puede añadir a la Enfermería, en la conducción de la asistencia individualizada a los pacientes bajo sus cuidados.

Causes and Treatment Outcomes of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in 82 Adult Patients

BACKGROUND/AIMS: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are predominantly known as medication-induced diseases. However, at our institution, we have experienced more cases of non-drug-related SJS and TEN than expected. Therefore, we studied the difference between non-drug-related and drug-related SJS and TEN in terms of clinical characteristics and prognoses. METHODS: The etiologies, clinical characteristics, and treatment outcomes for 82 adult patients with SJS and TEN were retrospectively reviewed. RESULTS: A total of 71 patients (86.6%) were classified as having SJS, and the other 11 patients (13.4%) were classified as having TEN. Drug-related cases were more common (43, 52.4%) than non-drug-related cases (39, 47.6%). Anticonvulsants (12/82, 14.6%) and antibiotics (9/82, 11%) were the most common causative medications. Anemia (p = 0.017) and C-reactive protein of > or = 5 mg/dL (p = 0.026) were more common in the drug-related cases than in the non-drug-related cases. Intravenous steroid therapy was used as the main treatment regimen (70/82, 85.4%). Of the 82 patients, 8 (9.8%) died during the clinical course. A univariate analysis for mortality showed statistical significance for the following: kidney function abnormality, pneumonia, hemoglobin of < 10 g/dL, and combined underlying diseases. In a multivariate analysis, only pneumonia was statistically significant (odds ratio, 25.79; p = 0.009). CONCLUSIONS: Drugs were the most frequent cause of these diseases. However, non-drug-related causes also contributed to a significant proportion of cases. Physicians should keep this in mind when documenting patient history. In addition, early recognition and treatment may be important for better outcomes.

A abordagem dos aspectos atuais do eritema multiforme e a Odontologia / The approach of the current erythema multiforme and dentistry

O eritema multiforme é um processo inflamatório agudo, que pode ser desencadeado por diversos fatores. Apresenta lesões muco-cutâneas com características próprias da doença. É dever do cirurgião-dentista saber diagnosticá-lo e tratá-lo, uma vez que, na maior parte dos casos, as lesões são evidenciadas na mucosa oral, podendo inclusive, serem restritas a esta área. Por esse motivo, este trabalho se destina, através de uma revisão da literatura, elucidar aos cirurgiões-dentistas conhecimentos relacionados aos aspectos clínicos, etiológicos, prognósticos, tipos de tratamentos e proservação dos casos de eritema multiforme.

The objective of the current research is to add dental surgeons information related to erythema multiforme. By means of literary review, this paper presents clinical and histological aspects, as well as etiology, prognosis, treatments and maintenance treatments for some specific cases. Erythema multiforme is an acute inflammatory process triggered by several factors. Patients may present characteristic mucocutaneous lesions and most cases present oral mucosal lesions which can often be limited to this site. Thus, it is the dental surgeon to diagnose and treat it.

Uso de inmunoglobulina humana endovenosa en pacientes con necrolisis epidérmica tóxica y síndrome de sobreposición Stevens Johnson necrolisis tóxica epidérmica / Use of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis and Stevens-Johnson/toxic epidermal necrolysis overlap syndrome. Review of 15 cases

Background: Toxic epidemial necrolysis (TEN) is an acute adverse drug reaction, that has an unpredictable progression and a 30 percent mortality. The incidence of TEN in the general population is approximately 0.4 to 1.2 cases/million/year. It is characterized pathologically by keratinocyte apoptosis which leads to epidemial detachment. Keratinocyte apoptosis is triggered by activation of the Fas-FasL, pathway and could be prevented by the use of intravenous immunoglobulin (IVIG). Aim: To report the experience with the use of IVIG in TEN. Material and methods: Retrospective study of 15 patients with a diagnosis of Stevens-Johnson/TEN overlap (SJS/TEN) or TEN, that received a total dose of 23 ± 0.6 mg/kg of IVIG over aperiod of 3 to 4 days. The infusion was initiated during the first 24 hours after diagnosis and was associated with standard care for burn victims. Steroids were avoided if the patient was not in chronic steroidal therapy. Results: All patients responded to IVIG in a lapse of 46.4 ± 14.2 hours from the beginning of infusion. Eighty percent of patients survived, but one developed acute renal failure due to IVIG, and another became blind due to corneal opacities, a complication of TEN. Those who survived were discharged after a lapse of 19-8 ± 6.6 days from the beginning of the disease. Conclusions: Despite the lack of blind, multicentric and randomized trials, we agree with some international studies that IVIG is beneficial as a treatment for SSJ/NET and TEN.

A case report of toxic epidermal necrolysis associated with geotrichosis due to Geotrichum silvicola / 中华皮肤科杂志

Objective To report a case of toxic epidermal necrolysis associated with geotrichosis due to Geotrichum silvicola. Methods The exudates from the body surface, blood and urine of the patient were examined by microscopy and simultaneously inoculated onto the Sabouraud dextrose agar (SDA) medium. The isolate was examined by microscopy, PCR which amplified the D1/D2 domain of 26S rDNA, and gene sequencing. Homologous sequences were searched in the GenBank/EMBL/DDBJ/PD nucleotide sequence library, and the genetic relationship was analyzed with the genealogical tree. Results Microscopy of pus from the abscess on the dorsa of left hand revealed a lot of spores and a few hyphae, which were not observed in the blood or urine specimens. Meanwhile, whitish colonies were grown in all the three successive cultures of blood and urine specimens, rather than the exudates on the body surface. After itraconazole and garlicin were administered for one week, both microscopic exam and fungus culture were negative. Microscopic exam of the isolate showed arthrospores arranged in chains, budding spores and a few of hyphae. It was found that there was a one-base difference between our isolate (Hebei-1) and the isolate from kerion -like eruptions (Changzheng-1), and a four-base difference between our isolate and the reference Geotrichum silvicola strain as well, in the D1/D2 domain of 26S rDNA. This isolate was identified to be most close to Changzheng-1 in the phylogenetic tree. Conclusion The patient with toxic epidermal necrolysis is associated with geotrichosis due to Geotrichum silvicola.

High-dose intravenous immunoglobulin in the treatment of some severe skin diseases: a clinical analysis / 中华皮肤科杂志

Objective To analyze the therapeutic outcomes and adverse effects of high-dose intravenous immunoglobulin (hd-IVIg) in the treatment of some severe skin diseases (toxic epidermal necrolysis, drug hypersensitivity syndrome, connective tissue disease, autoimmune bullous disease, acute graft-versus-host disease). Methods Twenty-five cases of severe skin diseases were treated with hd-IVIg (0.4 g/kg/day for a course of 5 days). Results The therapeutic outcomes were different from each other. Of all the cases, 21 improved, especially those of acute onset of toxic epidermal necrolysis and drug hypersensitivity syndrome; 1 adult dermatomyositis and 2 elder bullous pemphigoid were not relieved. A patient with acute graft-versus-host disease died. Three patients presented with minor adverse effects (headache and blood pressure rising). Conclusions hd-IVIg is effective and safe in the treatment of some severe skin diseases. More importantly, it has a substantial effect on shortening disease course and decreasing the dosage of glucocorticoids and immunosuppressants as well as on preventing infections.