Necrolisis tóxica epidérmica en cuidados intensivos / Epidermal toxic necrolysis in intensive care

Se estudiaron dos pacientes ingresados en sala de cuidados intensivos polivalente, los cuales presentaron necrolisis tóxica epidémica (NTE), complicación poco frecuente que tiene una incidencia de 1 a 4 casos por millón de habitantes. La causa de la enfermedad en estos pacientes fue medicamentosa y los principales medicamentos implicados fueron anfotericin B y ciprofloxacino. Una de las cuestiones que hace interesante el reporte de estas reacciones adversas es que se trata del primer caso de NTE relacionada con el uso de Anfotericin B Liposomal que se reporta en Cuba y en el resto de la comunidad científica. El diagnóstico se confirmó por biopsia de piel, ambos pacientes presentaron un riesgo de muerte, según índice de Scorten, mayor al 50 por ciento. Por lo infrecuente que es el tratamiento de esta afección en una sala de cuidados intensivos polivalentes, por lo raro que es ver esta enfermedad en relación a estos antibióticos antes mencionados, por lo dramático del cuadro clínico y por la evolución tórpida de los pacientes, se realizó esta investigación con el objetivo de transmitir la experiencia y el conocimiento a la comunidad médica y científica en general y a los profesionales que laboran en las salas de terapia intensiva polivalente en particular(AU)

Two patients admitted to a multipurpose intensive care ward were studied. They showed epidemic toxic necrolysis (NTE), a rare complication that has an incidence of 1 to 4 cases per million inhabitants. Drugs were the cause of the disease in these patients and the main drugs involved were amphotericin B and ciprofloxacin. This is the first case of NTE related to the use of Liposomal Amphotericin B reported in Cuba and in the rest of the scientific community, which makes it interesting. The diagnosis was confirmed by skin biopsy, both patients had risk of death, according to the Scorten index, higher than 50 percent. This research was carried out with the aim of communicating the experience and knowledge to the medical and scientific community in general and to the professionals who work in multipurpose intensive care rooms in particular, since this condition is rare in a multipurpose intensive care room, it is rare in relation to the aforementioned antibiotics, its dramatic clinical status and the torpid evolution of patients(AU)

Manifestações e tratamento da necrólise epidérmica tóxica e da síndrome de Stevens Johnson / Manifestations and treatment of toxic epidermic necrolysis and Stevens Johnson's syndrome / Manifestaciones y tratamiento de la necrolisis epidérmica tóxica y del síndrome de Stevens Johnson

Objetivo: identificar as manifestações clínicas da necrólise epidérmica tóxica (NET) e síndrome de Stevens Johnson (SSJ). Método: trata-se de uma revisão narrativa. A busca dos artigos utilizou a ferramenta Publish or Perish, que ranqueia os trabalhos com base no número de citações recebidas. Foram realizadas duas buscas, pois apesar das doenças se relacionarem, possuem diagnósticos diferentes. Na primeira, os descritores utilizados foram: "necrólise epidérmica tóxica" e "manifestações clínicas", e na segunda os descritores foram: "Síndrome de Stevens-Johnson" e "manifestações clínicas". Resultados: após a leitura dos 12 artigos selecionados, entendeu-se que a patogênese da necrólise epidérmica tóxica e Síndrome de Stevens Johnson se dá pela hipersensibilidade tardia a fármacos. As manifestações clínicas se dão pelo aparecimento do eritema cutâneo com formação de máculas, pápulas, vesículas e bolhas associadas ou isoladas, como placas de urticária ou eritema extenso. Na NET é possível notar desprendimento extenso da epiderme maior que 30% da superfície corpórea, conhecido como sinal de Nikolsky, com acometimento de mucosas. Conclusão: A NET e SSJ são farmacodermias graves, com baixas incidências, mas elevada mortalidade. O reconhecimento precoce das doenças e a retirada do fármaco causador são essenciais para conduzir o tratamento, diminuindo por sua vez a taxa de mortalidade.(AU)

Objective: to identify the clinical manifestations of toxic epidermal necrolysis (TEN) and Stevens Johnson syndrome (SJS). Method: the articles search was done using the Publish or Perish computational tool, which ranks the articles based on the number of citations. Two separate searches were performed, because although the diseases are related, they have different diagnoses. In the first, the descriptors used were "Toxic Epidermal Necrolysis" and "clinical manifestations", and in the second the descriptors were "Stevens-Johnson Syndrome" and "clinical manifestations". Results: in total, 12 articles constituted the present revision. It was understood that the pathogenesis of TEN and SJS is due to late drugs hypersensitivity. The clinical manifestations are due to the appearance of cutaneous erythema with the formation of macules, papules, vesicles and associated or isolated blisters, such as urticaria plaques or extensive erythema. In the TEN it is possible to notice extensive detachment of the epidermis greater than 30% of the body surface, known as Nikolsky's signal, with mucous involvement. Conclusion: TEN and SJS are serious skin diseases, with low incidences but high mortality. Early recognition of disease and withdrawal of the causative drug are essential for conducting treatment, thus decreasing the mortality rate. Descriptors: Nursing; Dermatology; Signs and Symptoms; Treatment; Health Management.(AU)

Objectivo: identificar las manifestaciones clínicas de la necrólisis epidérmica tóxica (NET) y el síndrome de Stevens Johnson (SSJ). Método: la selección de los artículos consideró el número de citas recibidas por otras publicaciones. Se realizaron dos búsquedas, pues a pesar de las enfermedades se relacionan, poseen diferentes diagnósticos. En la primera, los descriptores utilizados fueron: "Toxic Epidermal Necrolysis" y "clinical manifestations", y en los segundos los descriptores fueron: "Stevens-Johnson Syndrome" y "clinical manifestations". Resultados: en total, 12 artículos constituyeron la presente revisión. Se ha comprobado que la patogénesis de TEN y SJS se debe a las drogas de larga duración. Las manifestaciones clínicas se deben a la apariencia de cutánea erythema con la formación de macules, papules, vesicles y asociados, o blister, tales como urticaria plaquetas o extensión erythema. En el TEN es posible que tenga un detalle detallado de las epidermis mayor que el 30% de la superficie del cuerpo, conocidas la Nikolsky de la señal, con mucous. Conclusión: la NET y SSJ son farmacodermias graves, con bajas incidencias pero elevada mortalidad. El reconocimiento precoz de las enfermedades y la retirada del fármaco causante son esenciales para conducir el tratamiento, disminuyendo a su vez la tasa de mortalidad. Descriptores: Enfermería; Dermatología; Signos y Síntomas; Tratamiento; Gestión en Salud.(AU)

A systematic review of the drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in Indian population.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare severe cutaneous drug reactions. No large scale epidemiological data are available for this disorder in India. Aims: To carry out a systematic review of the published evidence of the drug-induced SJS and TEN in Indian population. Methods: Publications from 1995 to 2011 describing SJS and TEN in Indian population were searched in PubMed, MEDLINE, EMBASE and UK PUBMED Central electronic databases. Data were collected for the causative drugs and other clinical characteristics of SJS and TEN from the selected studies.Results: From 225 references, 10 references were included as per selection criteria. The major causative drugs were antimicrobials (37.27%), anti-epileptics (35.73%) and non-steroidal anti-inflammatory drugs (15.93%). Carbamazepine (18.25%), phenytoin (13.37%), fluoroquinolones (8.48%) and paracetamol (6.17%) were most commonly implicated drugs. Regional differences were observed for fluoroquinolones, sulfa drugs and carbamazepine. Total 62.96% of patients showed systemic complications. Most common complications were ocular (40.29%) and septicemia (17.65%). Higher mortality was observed for TEN as compared to SJS (odd ratio-7.19; 95% confidence interval (CI) 1.62-31.92; p = 0.0023). Observed mortality is higher than expected as per SCORTEN score 3. Duration of hospital stay was significantly higher in TEN (20.6 days; 95% CI 14.4-26.8) as compared to SJS (9.7 days; 95% CI 5.8-13.6; p = 0.020). Cost of management was significantly higher in TEN (Rs. 7910; 95% CI 5672-10147; p < 0.0001) as compared to SJS (Rs 2460; 95% CI 1762-3158). No statistical data were described for steroid use in the studies included. Conclusion: Carbamazepine, phenytoin, fluoroquinolones and paracetamol were the major causative drugs. TEN is showing higher mortality, morbidity and economic burden than SJS.

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.

SCORTEN: does it need modification?

BACKGROUND: Toxic epidermal necrolysis (TEN) is a drug induced acute life threatening condition with mortality ranging from about 15 to 60%. A 'severity of illness' score termed as SCORTEN has been developed to predict mortality in TEN cases at the time of admission. It is calculated by giving one point for each of predetermined seven variables, evaluated during first 24 hours of admission. Total score ranging from 1-7 predicts a probability of mortality from 0.03 to 0.90. AIM: A prospective study was conducted to analyze efficacy of 'SCORTEN' in TEN cases to predict mortality during their management. METHODS: All cases of TEN reporting for management to the hospital were assessed using 'SCORTEN' on day one and day five to predict probable mortality, this data was then compared with ultimate outcome. RESULTS: During the study period, we treated 10 cases of TEN, all induced by drugs, patient's age ranging from 03 to 70 years and body surface area (BSA) involvement from 10 to 95%. Three cases succumbed to death. These cases were analyzed with SCORTEN to predict probability of mortality at the time of admission and day five. We encountered some variations from the original study. It was observed that if patients are analyzed with SCORTEN on a daily/alternate day basis, it will serve as a better predictor of mortality. CONCLUSION: Body surface area (BSA) involvement and age probably need more weightage in calculations. Besides malignancy, tuberculosis and pre-existing diabetes also need to be included while predicting mortality.

Some unusual and severe forms of adverse drug reactions: a call for setting adverse drug reaction monitoring centres.

Thirty four patients aged 14 to 65 years (18 males and 16 females) admitted to the University Hospital with various unusual and severe forms of adverse drug reactions were studied. It comprised of toxic epidermal necrolysis in 8 patients, systemic vasculitis in 7 of which 3 patients had gangrene of fingers and/or toes, severe erosive gastritis in 9 patients, Stevens-Johnson syndrome in 7 patients, thrombocytopenic purpura in 2 patients and generalised convulsions in 1 patient. Various drugs responsible for causing these adverse drug reactions included antibacterials, antimalarials, anticonvulsants, antituberculars and nonsteroidal anti-inflammatory drugs. Most of the patients recovered. However, 5 of the 8 patients having toxic epidermal necrolysis died of which 2 patients had developed tetanus as a preterminal event. In view of ongoing addition of newer drugs to the therapeutic armamentarium and an increasing incidence of various unusual and severe forms of adverse drug reactions, it is our contention that a separate adverse drug reaction monitoring cell should be established in every hospital setting.

Síndrome de Stevens Johnson: reporte de 2 casos / Stevens-Johnson syndrome: report of 2 cases

Se reportan dos casos del síndrome de Stevens-Johnson en una paciente femenina de 25 años y un masculino de 17 años de edad. Ambos con la misma enfermedad pero con diferente grado de afección-, secundario a medicamentos con buena respuesta al tratamiento sintomático