scholarly journals An update on diagnosis and treatment of toxic epidermal necrolysis / Novine u dijagnostici i lečenju toksične epidermalne nekrolize

2011 ◽  
Vol 3 (2) ◽  
pp. 53-64
Author(s):  
Lidija Kandolf-Sekulović
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Case Reports ◽  
Supportive Therapy ◽  
Serum Levels ◽  
Intravenous Immunoglobulins ◽  
Treatment Modalities ◽  
Prognostic Indicators ◽  
Multiple Drug ◽  
High Dose ◽  
Therapeutic Effects

Abstract Toxic epidermal necrolysis is an idiosyncratic drug reaction which manifests with extensive epidermal detachment due to the massive keratinocyte apoptosis, mucous membrane involvement, and potentially lethal outcome. It is caused by adverse reactions to drugs, mostly idiosyncratic, unpredictable and independent of the applied dose, which develops 7-21 days after initiation of the drug, and is most commonly caused by the following drugs: sulfonamides, allopurinol, carbamazepine, phenobarbitone, phenytoin and oxycam group of nonsteroidal anti-inflammatory drugs. The treatment outcome depends on several factors, while older age, multiple drug use, late exclusion of the drug inducing toxic epidermal necrolysis, raised serum levels of urea, creatinine and cytopenia are poor prognostic indicators which are rated in SCORTEN scoring which proved to be of great help in the assessment of disease outcome. The basic approach to the treatment is early diagnosis, immediate suspension of the probable inducing drug, and emergency transport to the closest burn center, since treatment in burn units is associated with a lower risk of infection and mortality of these patients. Exclusion of the drug that induced toxic epidermal necrolysis, and supportive therapy, is the first and only therapy for which there is a consensus in different centers. Various forms of adjuvant therapy are also applied: in France, supportive therapy is a standard of care, in Germany it is short-term use of high-dose corticosteroids, while in USA, in the last decade high-dose intravenous immunoglobulins are the most widely accepted treatment modalities. Case reports and small patients’ series described therapeutic effects of plasmapheresis, cyclosporine and other immunosuppressants. In conclusion, elimination of the possible causal agent, rapid transport to the burn unit, and multidisciplinary approach to treatment are of utmost importance for favorable outcome of the disease with 20-30% mortality rate. An update on diagnosis and the treatment of toxic epidermal necrolysis is provided in this review.

High-Dose Intravenous Immunoglobulins in the Treatment of Toxic Epidermal Necrolysis: An Asian Series

2005 ◽  
Vol 32 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Audrey W. Tan ◽  
Bernard Y. Thong ◽  
Leonard W. Yip ◽  
Hiok Hee Chng ◽  
See Ket Ng
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Intravenous Immunoglobulins ◽  
High Dose

scholarly journals Combination of High-Dose Intravenous Immunoglobulins and Itraconozole in Treating Chronic Mycotic Demyelinating Optic Neuritis

2003 ◽  
Vol 3 ◽  
pp. 640-646 ◽  
Author(s):  
Andrew W. Campbell ◽  
Ebere C. Anyanwu ◽  
Aristo Vojdani
Keyword(s):  
Optic Neuritis ◽  
Chronic Exposure ◽  
Health Effect ◽  
Intravenous Immunoglobulins ◽  
Emerging Diseases ◽  
Treatment Modalities ◽  
White Female ◽  
High Dose ◽  
Visual Defects ◽  
Therapeutic Services

Mycotic demyelinating optic neuritis is a neurological disorder of the visual system caused by mycotoxins released by indoor toxic molds. Although the health effect of indoor toxic mold on the population worldwide is now one of the �emerging diseases�, its involvement in chronic demyelinating optic neuritis has not been reported. Most of the neurological and immunologic abnormalities associated with toxic mold mycotoxins are very difficult to treat successfully, especially neural demyelination of the central and peripheral nervous systems. This paper presents the case of a 42-year-old white female, in whom chronic demyelinating optic neuritis with persistent visual defects due to chronic exposure to toxic molds was diagnosed at the age of 34 years. In spite of all the therapeutic services given to her for over 8 years, her illness persisted and was difficult to treat. However, we successfully treated her with a combination of intravenous immune globulin (IVIG) and itraconozole (Sporanox) when all other treatment modalities failed. This is probably the first report where persistent toxic mold-induced neurological and immunologic disorders were successfully treated with a combination of itraconozole and IVIG.

Skin Reactions Due to Antiepileptic Drugs: Several Case-Reports with Long-Term Follow-up

2003 ◽  
Vol 16 (1) ◽  
pp. 89-93 ◽  
Author(s):  
C. Feliciani ◽  
A. Verrotti ◽  
G. Coscione ◽  
P. Toto ◽  
F. Morelli ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Antiepileptic Drugs ◽  
Case Reports ◽  
Complementary Therapy ◽  
Hypersensitivity Syndrome ◽  
Intravenous Immunoglobulins ◽  
Clinical Findings ◽  
Skin Reactions ◽  
First Choice ◽  
Cutaneous Reactions

In this study, the clinical findings and management of allergic skin reactions induced by the most used antiepileptic drugs, Lamotrigine (LMT) and Carbamazepine (CBZ), were evaluated. Lamotrigine is an antiepileptic drug recently released in several countries; it is effective for a variety of seizure types in adults and children, both as an add-on agent and in monotherapy, and it is generally well tolerated. Clinical and epidemiologic evidence suggest serious cutaneous reactions to antiepileptic drugs are more likely to occur during the first 8 weeks and they appear to increase when drugs are administered with other anticonvulsants, such as valproate (VPA). We selected 10 patients who presented an idiosyncratic skin rash when treated with carbamazepine (8 patients) and lamotrigine (2 patients) administered as monotherapy, and we followed up on these patients for several years. Seven reactions were mild/severe cutaneous eruptions; one Toxic Epidermal Necrolysis, a case of Stevens-Johnson and a case of Hypersensitivity Syndrome. All severe skin drug reactions were induced by Carbamazepine. In five patients the AEDs were ceased abruptly (sometimes with the administration of a different molecule), tapered in four and continued unchanged in one. We conclude that the discontinuation of the drug with substitution with another is the most effective treatment and that corticosteroids are helpful in mild cutaneous reactions, while in severe skin reactions, such as Toxic Epidermal Necrolysis, corticosteroids are only a complementary therapy since intravenous immunoglobulins are the first choice treatment.

A Retrospective Comparative Analysis of Factors Affecting the Decision and Outcome of Initial Intravenous Immunoglobulin Alone or Intravenous Immunoglobulin Plus Methylprednisolone Use in Children With the Multisystem Inflammatory Syndrome

2021 ◽  
Author(s):  
İlker Devrim ◽  
Elif Böncüoğlu ◽  
Elif Kıymet ◽  
Şahika Şahinkaya ◽  
Miray Yılmaz Çelebi ◽  
...  
Keyword(s):  
Intravenous Immunoglobulin ◽  
Pediatric Intensive Care ◽  
Serum Levels ◽  
Intravenous Immunoglobulins ◽  
Treatment Modalities ◽  
Double Blind ◽  
Factors Affecting ◽  
Group I ◽  
Group Ii ◽  
Inflammatory Syndrome

Abstract Background: Use of intravenous immunoglobulins (IVIG) with or without methylprednisolone is the most preferred therapeutic strategy for the multisystem inflammatory syndrome in children (MIS-C). This study aimed to compare the use of IVIG plus methylprednisolone vs IVIG alone in children with MIS-C. Methods: This retrospective cohort study was conducted during the period between April 1, 2020, and November 1, 2021. All children with MIS-C were included in the study. The patients were divided in two groups according to whether they were administered IVIG alone or IVIG plus methylprednisolone as an initial treatment for MIS-C. While the patients in group I were administered IVIG with a dosage of 2 gr/kg, the patients in group II were administered IVIG (dosage of 2 gr/kg) plus methylprednisolone (2 mg/kg/day). The re-occurrence of fever, duration of hospital stay, admission to pediatric intensive care unit were compared between these two groups. Results: A cohort of 91 patients under 18 years old and diagnosed as MIS-C was included in the study. Of these patients, 42 (46.2%) were in IVIG alone group. (group I) and 49 (53.8%) were in IVIG plus methylprednisolone (group II). The ratio of severe MIS-C was 36.7% for patients in the group II and significantly higher compared to the rate of severe MIS-C patients in the group I (9.5%) (p=0.01). The rate of hypotension was significantly higher in the group II (30.6 %) compared to the group I (9.5%) (p=0.014). Moreover, the mean serum levels of C-reactive protein were significantly higher for the patients in group II. The re-occurrence of fever was 26.5% in the group II and 33.3% in the group, however this difference was not statistically significant (p>0.05). Conclusions: The decision of the treatment choice of patients with MIS-C should be individually evaluated. In the clinically severe MIS-C patients who were with hypotension, and/or admitted to PICU should be treated with IVIG plus methylprednisolone. However, randomized double-blind studies are required for the treatment modalities of children with MIS-C.

Successful second ITI with factor IX and combined immunosuppressive therapy

2014 ◽  
Vol 34 (S 01) ◽  
pp. S5-S8 ◽  
Author(s):  
R. Schneppenheim ◽  
J. Schrum ◽  
C. Bokemeyer ◽  
F. Langer ◽  
K. Holstein
Keyword(s):  
Nephrotic Syndrome ◽  
Immunosuppressive Therapy ◽  
Case Reports ◽  
Intravenous Immunoglobulins ◽  
Factor Ix ◽  
High Dose ◽  
Allergic Reactions ◽  
Success Rates ◽  
Skin Reactions ◽  
Haemophilia B

SummaryImmune tolerance induction (ITI) in patients with haemophilia B and inhibitors may be complicated by anaphylactic reactions and nephrotic syndrome with lower success rates than in haemophilia A (25% vs. 50–90%). According to case reports, immunosuppressive therapy in addition to high doses of factor IX (FIX) appears to be promising. We report an 18-year-old patient with severe haemophilia B and a FIX inhibitor with a maximum titre of 2.6 Bethesda units and allergic skin reactions to FIX infusions. At 5 years of age, this patient already had a FIX inhibitor with allergic reactions to FIX and activated prothrombin complex concentrate. ITI at 11 years of age with high-dose FIX, de-xamethasone, rituximab, mycophenolate mofetil and intravenous immunoglobulins had induced a sustained response until the current presentation.The patient was restarted on the same ITI regimen with aforementioned immunosup-pressants, which were initiated one week before high-dose FIX. No allergic reactions, nephrotic syndrome or serious infection occurred during ITI. The FIX inhibitor was unde-tectable after five weeks of treatment and remained so until 19 months of follow-up.

Stevens–Johnson syndrome and toxic epidermal necrolysis: a 10-year experience in a burns unit

2021 ◽  
Vol 30 (6) ◽  
pp. 492-496
Author(s):  
Khosrow S Houschyar ◽  
Christian Tapking ◽  
Mimi R Borrelli ◽  
Ina Nietzschmann ◽  
Behrus Puladi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Toxic Epidermal Necrolysis ◽  
Retrospective Review ◽  
Mortality Rates ◽  
Intravenous Immunoglobulins ◽  
Stevens Johnson Syndrome ◽  
Control Group ◽  
High Dose ◽  
Johnson Syndrome ◽  
Patient Reported

Objective: Stevens-Johnson syndrome (SJS) and its more severe counterpart, toxic epidermal necrolysis (TEN), are skin hypersensitivity reactions defined by epidermal blistering and necrosis. The exact pathophysiology of SJS/TEN is yet to be deciphered, but a number of risk factors have been identified including adverse drug reactions. The diagnosis of SJS/TEN is made on a clinical basis, and treatment consists of supportive care and occasionally immunosuppressants, such as cyclosporin, high-dose intravenous immunoglobulins and/or corticosteroids. Mortality rates can reach 20–25% in adults but are reduced with early intervention. To identify optimal treatment regimens, to better understand the patient cohort affected, and to help identify key risk factors for mortality, we report our experience with the treatment and management of SJS/TEN patients. Methods: A retrospective review of consecutive patients with SJS and/or TEN admitted to a single burns centre in Germany, between 2008 and 2018, was conducted. The primary outcomes of demographics, clinical course, treatment and patient-reported outcomes were recorded and compared with a control group of patients with burns without a diagnosis of SJS/TEN. Results: A total of 23 patients with SJS/TEN met the inclusion criteria: 17 (74%) with TEN; four (17%) with SJS/TEN overlap; and two (9%) with SJS. Of the patients, 14 (61%) were female and nine (39%) were male. Patient age ranged from 32–78 years (mean: 52 years). A matched cohort of 23 patients with burns served as the control group. All patients received standard of care with a multidisciplinary team. Compared with the control group, SJS/TEN patients had higher mortality rates (n=6, 26% versus n=8, 35%, respectively). The average age of death was 69 years in SJS/TEN patients versus 63 years in control group patients. Age and SCORTEN scores were significant predictors of mortality. Conclusions: SJS and TEN are rare but extreme reactions of the skin and mucosa, associated with high disease mortality rates. This 10-year single-centre retrospective review contributes to the bank of information for reviews evaluating the management of SJS/TEN patients.

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