TEN mimics: Classification and practical approach to toxic epidermal necrolysis-like dermatoses.

Toxic epidermal necrolysis (TEN) is an acute life-threatening dermatologic emergency. However, many dermatoses can present with a TEN-like eruption. Those "TEN-mimics" are a true diagnostic challenge and an alarming differential diagnosis to such a serious condition. Herein, we will expose and classify the landscape of TEN-mimics. Also, the key differentiating clinical and/or laboratory points will be highlighted to help an accurate diagnosis of either a TEN or a TEN-like presentation.

Comparison of effectiveness of interventions in reducing mortality in patients of toxic epidermal necrolysis: A network meta-analysis.

BACKGROUND: Limited evidence is available about effectiveness and choice of immunomodulating treatment modalities for toxic epidermal necrolysis (TEN). AIMS: To compare the effectiveness of interventions to reduce mortality in patients of toxic epidermal necrolysis through network meta-analysis. METHODS: Studies were retrieved using PubMed, Google Scholar and Cochrane Database of Systematic Reviews from inception to September 18, 2018. Only English language articles were considered. Observational and randomized controlled studies having ≥ 5 TEN patients in each intervention arm were included. Two investigators independently extracted study characteristics, intervention details and mortality data. Bayesian network meta-analysis was performed using the Markov chain Monte Carlo (MCMC) approach through the random effect model. The ranking analysis was done to provide a hierarchy of interventions. The consistency between direct and indirect evidence was assessed through node spit analysis. The primary outcome was to compare the mortality [Odds ratio OR (95% credibility interval CrI)] among all treatment modalities of TEN. RESULTS: Twenty-four studies satisfying the selection criteria were included. The network analysis showed improved survival with cyclosporine as compared to supportive care [OR- 0.19 (95% CrI: 0.05, 0.59)] and intravenous immunoglobulin [OR- 0.21 (95% CrI: 0.05, 0.76)]. The hierarchy of treatments based on "surface under the cumulative ranking curves" (SUCRA) value were cyclosporine (0.93), steroid+intravenous immunoglobulin (0.76), etanercept (0.59), steroids (0.46), intravenous immunoglobulin (0.40), supportive care (0.34) and thalidomide (0.02). No inconsistencies between direct and indirect estimates were observed for any of the treatment pairs. LIMITATIONS: Evidence is mainly based on retrospective studies. CONCLUSION: The use of cyclosporine can reduce mortality in TEN patients. Other promising immunomodulators could be steroid+intravenous immunoglobulin combination and etanercept.

Oseltamivir-induced toxic epidermal necrolysis in a patient with Cushing's disease.

We report a case of a patient with Cushing's disease with oseltamivir-induced toxic epidermal necrolysis, who was treated with cyclosporine with favorable evolution. There is only one case reported of Cushing's disease and toxic epidermal necrolysis and very few oseltamivir-induced toxic epidermal necrolysis cases in literature. This report also discusses the role that the preexisting hypercortisolism condition may have played in the development and favorable resolution of the toxic epidermal necrolysis.

Guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis: An Indian perspective.

BACKGROUND: Stevens-Johnson syndrome and toxic epidermal necrolysis are severe, life-threatening mucocutaneous adverse drug reactions with a high morbidity and mortality that require immediate medical care. The various immunomodulatory treatments include systemic corticosteroids, cyclosporine, intravenous immunoglobulin, cyclophosphamide, plasmapheresis and tumor necrosis factor-α inhibitors. AIM: The ideal therapy of Stevens-Johnson syndrome/toxic epidermal necrolysis still remains a matter of debate as there are only a limited number of studies of good quality comparing the usefulness of different specific treatments. The aim of this article is to comprehensively review the published medical literature and frame management guidelines suitable in the Indian perspective. METHODS: The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL) assigned the task of preparing these guidelines to its special interest group on cutaneous adverse drug reactions. The group performed a comprehensive English language literature search for management options in Stevens-Johnson syndrome/toxic epidermal necrolysis across multiple databases (PubMed, EMBASE, MEDLINE and Cochrane) for keywords (alone and in combination) and MeSH items such as "guidelines," "Stevens-Johnson syndrome," "toxic epidermal necrolysis," "corticosteroids," "intravenous immunoglobulin," "cyclosporine" and "management." The available evidence was evaluated using the strength of recommendation taxonomy and graded using a three-point scale. A draft of clinical recommendations was developed on the best available evidence which was also scrutinized and critically evaluated by the IADVL Academy of Dermatology. Based on the inputs received, this final consensus statement was prepared. RESULTS: A total of 104 articles (meta-analyses, prospective and retrospective studies, reviews [including chapters in books], previous guidelines [including Indian guidelines of 2006] and case series) were critically evaluated and the evidence thus gathered was used in the preparation of these guidelines. RECOMMENDATIONS: This expert group recommends prompt withdrawal of the culprit drug, meticulous supportive care, and judicious and early (preferably within 72 h) initiation of moderate to high doses of oral or parenteral corticosteroids (prednisolone 1-2 mg/kg/day or equivalent), tapered rapidly within 7-10 days. Cyclosporine (3-5 mg/kg/day) for 10-14 days may also be used either alone, or in combination with corticosteroids. Owing to the systemic nature of the disease, a multidisciplinary approach in the management of these patients is helpful.

Toxic epidermal necrolysis / Toxic epidermal necrolysis

Toxic epidermal necrolysis (TEN) is an unpredictable, life-threatening drug reaction associated with a 30 po cento mortality. Massive keratinocyte apoptosis is the hallmark of TEN. Cytotoxic T lymphocytes appear to be the main effector cells and there is experimental evidence for involvement of both the Fas-Fas ligand and perforin/granzyme pathways. Optimal treatment for these patients remains to be clarified. Discontinuation of the offending drug and prompt referral to a burn unit are generally agreed upon steps. Beyond that, however, considerable controversy exists. Evidence both pro and con exists for the use of IVIG, systemic corticosteroid, and other measures. There is also evidence suggesting that combination therapies may be of value. All the clinical data, however, is anecdotal or based on observational or retrospective studies. Definitive answers are not yet available. Given the rarity of TEN and the large number of patients required for a study to be statistically meaningful, placebo controlled trials are logistically difficult to accomplish. The absence of an animal model further hampers research into this condition. This article reviews recent data concerning clinical presentation, pathogenesis and treatment of TEN. LEARNING OBJECTIVES: At the conclusion of this learning activity, participants should have acquired a more comprehensive knowledge of our current understanding of the classification, clinical presentation, etiology, pathophysiology, prognosis, and treatment of TEN.

Exploiting the igloo principle and greenhouse effect to regulate humidity and temperature.

BACKGROUND: Toxic epidermal necrolysis can be fatal and nursing care with careful monitoring of temperature and humidity can improve survival rate. We adapted the greenhouse and igloo principle using a common hood to monitor the temperature and humidity. METHODS: A small heater with a regulator was placed in a mini hood and temperature was recorded inside the uncovered hood and hood covered with green cloth and aluminium foil separately. The regular hood was placed over a volunteer and the temperature was measured inside the open hood and hood covered with green cloth and aluminium foil separately. The relative humidity was also monitored using Zeal mercury dry--wet bulb hygrometer. RESULTS: Temperature increase was most marked in the foil-covered hood followed by cloth-covered hood, both with the heater and the volunteer. Similarly, in the volunteer study, the humidity was best maintained inside the aluminium foil-covered hood. CONCLUSION: We recommend the use of regular hood with suitable cover to monitor the humidity and temperature of patients with toxic epidermal necrolysis.

Achieving asepsis of banana leaves for the management of toxic epidermal necrolysis.

BACKGROUND: Banana leaf is used in many centers in India during the care of patients with toxic epidermal necrolysis (TEN) and other extensive blistering disorders. Sepsis is an important cause of death in TEN patients and use of banana leaf may be a source of such infection. AIMS: We conducted this study to detect the bacterial flora of the banana leaf and to examine various methods of rendering the leaf aseptic. METHODS: Five pieces of banana leaf, 2 x 2 cm in size, were cultured separately in blood agar as follows: One piece was heated over a flame and one was soaked in boiling water and one was autoclaved. Methylated spirit was applied over one piece and ignited. One piece was placed on the media, 'as is.' The Petri dishes were incubated examined after 48 h. RESULTS: All the pieces except the autoclaved specimen of the leaf grew coagulase-negative staphylococci (CONS) when aseptic precautions were not maintained and aerobic spore bearers when all aseptic measures were subsequently instituted during the procedure. CONCLUSION: We recommend measures to prevent possible transmission of bacterial infection by the leaf. Autoclaved and aseptically handled banana leaves may be used to reduce chance of infection in the treatment of TEN.

Efficacy of low dose intravenous immunoglobulins in children with toxic epidermal necrolysis: an open uncontrolled study.

BACKGROUND: High dose intravenous immunoglobulins (IVIG) have emerged as a promising new therapy for treating the rare but potentially fatal drug reaction toxic epidermal necrolysis (TEN). Experimental in vitro studies support the view that IVIG can block the fas-fas ligand mediated apoptosis in TEN. METHODS: Ten pediatric patients of TEN were treated with IVIG (0.05 - 0.1 gm/kg/day) along with antibiotics and supportive care. RESULTS: Patients with 67% of mean body surface area of involvement showed an average of 2.1 days for arrest of progression of lesions and 8.1 days for complete reepithelization. There was no mortality. CONCLUSIONS: Low dose IVIG appears to be a safe and effective treatment for TEN in children. Randomized trials are needed to further evaluate the efficacy of IVIG and compare it with other therapeutic modalities.