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.

Reação cutânea grave induzida por carbamazepina no tratamento da neuralgia pós-herpética: relato de caso / Reacción cutánea grave inducida por la carbamazepina en el tratamiento de la neuralgia postherpética: relato de caso / Severe carbamazepine-induced cutaneous reaction in the treatment of post-herpetic neuralgia: case report

JUSTIFICATIVA E OBJETIVOS: O herpes zoster tem como principal complicação a neuralgia pós-herpética (NPH). Utiliza-se para o tratamento a carbamazepina (CXB), um anticonvulsivante bem tolerado, porém frequentemente associado a reações Cutâneas graves, como, por exemplo, a síndrome de Stevens-Johnson (SSJ) e a necrólise epidérmica tóxica (NET). O objetivo deste trabalho é relatar um caso de SSJ/NET secundário ao uso de CBZ em paciente com NPH. RELATO DO CASO: Paciente do sexo feminino, com dor contínua em região torácica e dorso, intensa, em queimação, fisgada, choque, alteração de força de membro superior ipsilateral e sudorese. Apresentava lesões crostosas e eritematosas em região dorsal do tórax, com alodinia e disestesias em dermátomo acometido. Iniciou-se CBZ 300 mg.dia-1, amitriptilina (AMT) 12,5 mg à noite e infiltração com anestésico local na região afetada. Após 15 dias, referia mal-estar, febre, dores musculares e artralgias com rash cutâneo leve e inespecífico. Retirou-se a carbamazepina imediatamente. Uma semana depois, foi internada com urticária e exantema generalizados, erupções Cutâneas eritematosas, bolhosas e máculas purpúricas por todo o corpo. A impressão era de SSJ/NET induzida por carbamazepina. Houve progressiva piora do quadro, com aumento do número e do tamanho das lesões Cutâneas, além de rash eritematoso macular generalizado, áreas de necrose e erosões, com destacamento simétrico da epiderme em face, pescoço, tórax, dorso e membros acometendo mais de 50 por cento da área de superfície, além de envolvimento da mucosa bucal, conjuntival e genital com erosões vesiculares. Apresentou piora funcional progressiva, evoluindo com choque séptico e falência múltipla de órgãos, indo a óbito. CONCLUSÕES: A SSJ/NET é uma reação Cutânea grave com potencial para morbidade e mortalidade elevadas e que demanda intervenção rápida e tratamento adequado. Fica também o alerta para o uso da carbamazepina, que deve sempre ser supervisionado, ...

BACKGROUND AND OBJECTIVES: Post-herpetic neuralgia (PHN) is the main complication of herpes zoster. Carbamazepine (CBZ), a well-tolerated anticonvulsant, but frequently associated with severe cutaneous reactions, such as the Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is used in the treatment of this complication. The objective of this article was to report a case of SJS/TEN secondary to CBZ in a patient with PHN. CASE REPORT: This is a female patient with continuous severe, burning, chock-like pain in the thoracic region and dorsum associated with reduced strength in the ipsilateral upper limb and diaphoresis. She had crusty and erythematous lesions in the dorsal region of the thorax with allodynia and dysesthesia in the affected dermatome. She was treated with CBZ 300 mg.day-1, amitriptyline (AMT) 12.5 mg at bedtime, and infiltration with local anesthetic in the affected region. After 15 days, she developed malaise, fever, muscle pain, and arthralgia with a mild non-specific cutaneous rash. Carbamazepine was discontinued immediately. One week later, she was hospitalized with urticaria, generalized exanthema, erythematous cutaneous eruptions, bullae, and purpuric maculae all over her body. The impression was of carbamazepine-induced SJS/TEN. She evolved with progressive worsening of her symptoms, with increase in the number and size of cutaneous lesions, besides generalized erythematous macular rash, areas of necrosis, and erosions with symmetrical loosening of the epidermis in face, neck, thorax, dorsum, and limbs, affecting more that 50 percent of her body surface, besides involvement of buccal, conjunctival, and genital mucosa with vesicular erosions. She had progressive functional worsening, evolving to septic shock and multiple organ failure followed by death. CONCLUSIONS: Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous reaction with potential for elevated morbidity and mortality ...

JUSTIFICATIVA Y OBJETIVOS: El herpes zoster tiene como principal complicación la neuralgia postherpética (NPH). Para su tratamiento se usa la carbamazepina (CBZ), un anticonvulsivo bien tolerado, pero que sin embargo está a menudo asociado a reacciones cutáneas graves, como por ejemplo, el síndrome de Stevens-Johnson (SSJ) y la necrólisis epidérmica tóxica (NET). El objetivo de este trabajo es relatar un caso de SSJ/NET secundario al uso de CBZ en paciente con NPH. RELATO DEL CASO: Paciente del sexo femenino, con dolor continuo e intenso en la región torácica y dorso, ardor, punzada, descarga eléctrica, alteración de fuerza del miembro superior ipsilateral y sudoración. Presentaba lesiones de postillas y eritemas en la región dorsal del tórax, con alodinia y disestesias en el dermatoma acometido. Se inició CBZ 300 mg.día-1, amitriptilina (AMT)12,5 mg por la noche e infiltración con anestésico local en la región afectada. Después de 15 días, el paciente decía sentir un fuerte malestar, fiebre, dolores musculares y artralgias con rash cutáneo ligero e inespecífico. Se le retiró la carbamazepina inmediatamente. Una semana después fue ingresado con urticaria y exantema generalizados, erupciones cutáneas eritematosas, burbujas y marcas purpúricas por todo el cuerpo. La impresión era de SSJ/NET inducida por carbamazepina. Hubo un progresivo empeoramiento del cuadro, con aumento del número y del tamaño de las lesiones cutáneas, además de rash eritematoso macular generalizado, áreas de necrosis y erosiones simétricas de la epidermis en la cara, cuello, tórax, dorso y miembros, llegando a más del 50 por ciento del área de superficie, además de la involucración de la mucosa bucal, conjuntival y genital con erosiones vesiculares. Presentó un empeoramiento funcional progresivo, evolucionando con choque séptico y fracaso multiorgánico, lo que produjo finalmente su deceso. CONCLUSIONES: La SSJ/NET es una reacción cutánea grave con potencial para la ...

Occupational Skin Diseases in Korea

Skin disease is the most common occupational disease, but the reported number is small in Korea due to a difficulty of detection and diagnosis in time. We described various official statistics and data from occupational skin disease surveillance system, epidemiological surveys and cases published in scientific journals. Until 1981, 2,222 cases of occupational skin disease were reported by Korean employee's regular medical check-up, accounting for 4.9% of the total occupational diseases. There was no subsequent official statistics to figure out occupational skin diseases till 1998. From 1999, the Korea Occupational Safety and Health Agency (KOSHA) published the number of occupational skin diseases through the statistics of Cause Investigation for Industrial Accidents. A total of 301 cases were reported from 1999 to 2007. Recent one study showed the figures of compensated occupational skin diseases. Many of them belonged to daily-paid workers in the public service, especially forestry workers. Also, it described the interesting cases such as vitiligo and trichloroethylene-induced Stevens-Johnson Syndrome. Skin diseases are still important though the number of cases has decreased, and therefore it is recommended to grasp the status of occupational skin diseases through continuous surveillance system and to make policy protecting high-risk group.

Cutaneous drug reactions in patients admitted to the dermatology unit at the University Hospital of the West Indies, Kingston, Jamaica / Reacciones cutáneas medicamentosas en pacientes ingresados en la Unidad de Dermatología del Hospital Universitario de West Indies, Kingston, Jamaica

OBJECTIVE: Cutaneous reactions are among the most common adverse reactions to drugs. The purpose of this study is to examine the aetiology and outcome of cutaneous drug reactions among patients admitted to the Dermatology Ward at the University Hospital of the West Indies. SUBJECTS AND METHODS: This was a retrospective study looking at all patients who were admitted with a diagnosis of a cutaneous drug eruption from January 1, 1997 to December 31, 2005. Data included patient demographics, date of admission to hospital, duration of hospitalization and a detailed drug history including any previous episodes of drug sensitivity. All drugs reportedly ingested by the patients up to three months prior to their cutaneous reaction were documented and the period of time between drug ingestion and the appearance of skin lesions was also noted. Clinical diagnosis, co-morbidities, histopathological diagnosis, final outcome and all ensuing disabilities were noted. The data retrieved were collated and analyzed using SPSS 12.0. RESULTS: The results showed a female to male ratio of 2.2:1. The categories of drugs most commonly implicated were antimicrobials followed by anti-epileptic drugs and nonsteroidal anti-inflammatory drugs. The most common form of drug eruption requiring admission was the exanthematous drug eruption followed by erythema multiforme, toxic epidermal necrolysis and Stevens-Johnson syndrome. CONCLUSION: In general, the causative agents identified and the types of drug eruptions were similar to those found in previous studies. However, the anti-epileptic drugs, phenytoin and carbamazepine, ranked among the most commonly implicated drugs which differ significantly from other studies.

Las reacciones cutáneas se hallan entre las reacciones adversas más comunes frente a los medicamentos. El propósito de este estudio fue examinar la etiología y la evolución clínica de las reacciones cutáneas medicamentosas entre pacientes ingresados a la sala de dermatología en el Hospital Universitario de West Indies. MÉTODOS: Este es un estudio retrospectivo que pasa revista a todos los pacientes que fueron ingresados con diagnóstico de erupción cutánea desde el 1ero. de enero de 1997 al 31 de diciembre de 2005. RESULTADOS: Los resultados mostraron una proporción hembra-varón de 2.2:1. Las categorías de los medicamentos más frecuentemente implicados fueron los antimicrobianos, seguidos por los medicamentos antiepilépticos y los antiinflamatorios no esteroideos. La forma más común de erupción que requirió ingreso a causa de medicamentos, fue la erupción exantemática medicamentosa seguida por el eritema multiforme, la necrólisis epidérmica tóxica, y el síndrome de Stevens-Johnson. CONCLUSIÓN: En general, los agentes causativos identificados y los tipos de erupciones medicamentosas, fueron similares a los hallados en estudios previos. Sin embargo, los antiepilépticos conocidos como fenitoína y carbamazepina, estuvieron entre los medicamentos más comúnmente implicados, presentándose en tal sentido una diferencia significativa con los otros estudios.

Síndrome de Stevens-Johnson associada ao uso de antimicrobiano / Stevens-Johnson syndrome associated with antimicrobial agent

Dentre as reações medicamentosas graves, figuram como grandes representantes as doenças mucocutâneas imunologicamente mediadas. A síndrome de Stevens-Johnson, ou eritema multiforme maior, aparece como um distúrbio sistêmico com envolvimento de pele e membranas mucosas relacionado a diversos fatores, tais como infecções virais ou bacterianas e, principalmente, a administração de medicamentos, em geral analgésicos e antibióticos. O objetivo deste artigo é relatar o aparecimento de lesões vesículo-bolhosas ulcerativas em regiões de lábios, gengiva, língua e mucosa genital em um paciente de 26 anos, leucoderma, gênero masculino, em tratamento de infecção respiratória com sulfametoxazol-trimetropima, diagnosticado como síndrome de Stevens-Johnson

Among the severe reactions to medications, immune-mediated Mucocutaneous Disorders are widely represented. Steven-Johnson's syndrome, or great multiform erythema, appears as a systemic disturbance, involving the skin and mucous membranes, and is related to several factors, such as, viral or bacterial infections and particularly the administration of medicines, in general painkillers and antibiotics. The objective of this article is report the onset of ulcerative vesicle ûblister lesions in the regions of the lips, gums, tongue and genital mucosa membrane in a 26 year-old patient, a leukoderm man, being treated with sulfamethoxazole trimethoprim for a respiratory infection, after being diagnosed as having the Steven-Johnson syndrome.

Clinical study of cutaneous drug eruptions in 200 patients.

BACKGROUND: Cutaneous drug reactions are the most common adverse reactions attributed to drugs. Any skin disorder can be imitated, induced or aggravated by drugs. AIMS: The present study was carried out to determine the age, sex incidence and clinical pattern of drug eruptions, to recognize offending drugs (self medication or prescribed), to evaluate mortality and morbidity associated with drugs, to educate the patients, and to avoid self-administration of drugs and re-administration of the offending drugs. METHODS: The diagnosis of cutaneous drug reactions is mainly based on detailed history and correlation between drug intake and the onset of rash. Two hundred patients (112 males and 88 females) presenting with cutaneous drug reactions were studied. RESULTS: Fixed drug eruption was seen in 61 patients; others being urticaria and angioedema, morbilliform rash in 37, pruritus in 25, Stevens Johnson (SJ) syndrome in six, purpura in six, exfoliative dermatitis in five, photosensitivity in five, Toxic Epidermal Necrolysis in two, acneiform eruption in three, and erythema multiforme in two patients. The most frequently affected age group was 41-50 years, followed by the 21-30 and 31-40 years age groups. The youngest patient was one year old and the oldest was 80 years old. The period of development of lesions after the intake of drug(s) varies from 01-45 days. Cotrimoxazole was the offending drug in 26 cases, followed by Ibuprofen in 20 cases. CONCLUSIONS: Fixed drug eruption was the most common drug eruption seen. Cotrimoxazole was the most common cause of drug eruptions.

Stevens Johnson syndrome, toxic epidermal necrolysis and SJS-TEN overlap: a retrospective study of causative drugs and clinical outcome.

BACKGROUND AND AIMS: Stevens Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and SJS-TEN overlap are serious adverse cutaneous drug reactions. Drugs are often implicated in these reactions. METHODS: A retrospective analysis of inpatients' data with these dermatological diagnoses were carried out for three years, to study the causative drugs, clinical outcome, and mortality in these conditions. RESULTS: Thirty patients (15 TEN, nine SJS-TEN overlap, and six SJS) were admitted. In 21 cases, multiple drugs were implicated whereas single drugs were responsible in nine. Anticonvulsants (35.08%) were the most commonly implicated drugs followed by antibiotics (33.33%) and NSAIDS (24.56%). Twenty-five patients recovered whereas five died (four TEN, one SJS-TEN overlap). CONCLUSION: Anticonvulsants, antibiotics and NSAIDs were the most frequently implicated drugs. TEN causes higher mortality than both SJS and SJS-TEN overlap.

Síndrome de Stevens Johnson: una enfermedad habitualmente producida por medicamentos / Stevens Johnson's syndrome: a disease usually caused by drugs

El síndrome de Stevens Johnson, forma menor de la necrosis epidérmica tóxica, es una enfermedad grave, típicamente secundaria a medicamentos. Se describe el caso de un niño de 4 años que presentó un síndrome de Stevens Johnson con un compromiso cutáneo de 15%, probablemente secundario a carbamacepina. Establecer la relación causal entre un medicamento y un evento adverso exige un alto índice de sospecha y conocer la importancia de la patología inducida por medicamentos en la práctica clínica. La notificación de las sospechas de reacciones adversas a medicamentos es la herramienta fundamental para detectar señales de alerta sobre la seguridad de los mismos. Esta comunicación tiene como objetivos sensibilizar a los pediatras sobre la importancia y potencial gravedad de la patología inducida por medicamentos y revisar la conducta terapéutica frente al SSJ, una de las reacciones adversas graves más frecuentes.

Stevens Johnson Syndrome is a manifestation of toxic epidermal necrosis. It is a rare disease usually secondary to drugs. The case of a four-year old child who had 15% of cutaneous compromise, secondary to carbamacepine is presented. Drug induced diseases the establishment of a relationship between a drug and a side effect requires a high level of suspicion. Any case of probable drug induced side effect should be notified in order to establish the drug's safety. The objective of this paper is to highlight the importance of the topic and also to revise the treatment of Stevens Johnson Syndrome which is a severe and frequent adverse reaction.

Síndrome de Stevens-Johnson: relato de caso e revisão da literatura / Stevens-Johnson syndrome: case report and literature review

As reações cutâneas às drogas são as que geralmente ne­cessitam de internação hospitalar, por vezes em unidade de terapia intensiva ou de queimados, com observação minuciosa dos sinais vitais e função de órgãos internos. A síndrome de Stevens-Johnson é uma rara e grave reação cutânea e muco­sa, causada comumente por drogas, como as sulfonamidas, AINES, agentes anticonvulsivantes e alopurinol. Neste artigo, nós abordamos as características clínicas da síndrome de Ste­vens-Johnson, com o objetivo de facilitar o seu reconhecimen­to e tratamento de forma precoce.

Carbamazepine--the commonest cause of toxic epidermal necrolysis and Stevens-Johnson syndrome: a study of 7 years.

BACKGROUND: Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are a group of severe life threatening drug reactions. The drugs commonly implicated as the cause of these drug reactions vary depending on host factors and the prescription pattern of drugs in that particular area. AIM: The aim of the study was to find the drugs implicated as the cause of SJS/TEN in the patients admitted in the dermatology ward at the Medical College, Thrissur and to find the clinical outcome. METHODS: It was a retrospective study of 7 years from 1997 to 2004. The case records of all patients with a clinical diagnosis of TEN or SJS were studied in detail regarding the drugs implicated as the cause, the management and the clinical outcome. RESULTS: During the study period, 41 patients in the age group ranging from 12 to 72 years were treated as inpatients, of which 20 were males and 21 were females. The commonest drug implicated as the cause of SJS/TEN was carbamazepine (44%). The indication for carbamazepine was control of pain in more than 50% of the cases. Presence of a major systemic disease before the onset of SJS/TEN was associated with a bad prognosis. CONCLUSION: The increased use of carbamazepine, especially for control of pain, may be the reason for the increased incidence of SJS/TEN due to the same drug. Awareness about the drugs implicated in life threatening drug reactions will help physicians in preventing them by judicious use of the drugs.

Reacciones adversas medicamentosas graves: síndrome de Stevens-Johnson y necrólisis epidérmica tóxica / Severe adverse reactions: Stevens-Johnson syndrome and toxic skin necrolysis

Las reacciones cutáneas son las reacciones adversas medicamentosas más frecuentes ocurriendo en el 2 por cientode todos los tratamientos. El objetivo de este trabajo es realizaar una revisión dee los medicamentos que con mayor frecuencia causan el síndrome de Stevens-Johnson, la necrólisis epidérmica tóxica y señalar sus manifestaciones clínicas basados en la literatura y en los reportes del sistema nacional de farmacovigilancia. Especialmente nos interesa señalar estos síndromes como un problema relacionado con medicamentos, ya que algunos fármacos, muchos de ellos de uso prevalente, so el agente tiológico en la mayoría de los casos. La suspensión de aquellos no dedstinados a sostener una función vital es prioritaria ante la sospecha de una reacción cutánea severa. existen síntomas y signos que permiten al médico identificar los casos potencialmente graves. Los compuestos asociados con este tipo de reacciones sólo pueden ser identificados si los clínicos sospechan que existe una relación causa-efecto y posteriormente la informan. Es difícil conocer todo acerca de todos los medicamentos que prescribimos. Podemos acercarnos a esa meta si seleccionamos unos pocos para nuestra práctica clínica habitual.

Steven Johnson syndrome due to I.V Ceftriaxone--a case report.

Steven-Johnson syndrome (SJS) is a rare vesiculobullous disease characterized by an acute cutaneous eruption that ivolves the skin and mucous membranes including those of the oral cavity. A rare case of Steven-Johnson syndrome, an unexpected treatment response, in a 25-year-old female patient due to administration of intravenous Cefriaxone (1 gm), a third generation cephalosporin has been reported and literature reviewed.

Exantemas febriles no infecciosos / Non infectious febrile rash

Exantema es una erupción de la piel con lesiones de distinto tipo, configuración y disposición. El diagnóstico diferencial para pacientes febriles con exantema es extenso. Las posibles causas son enfermedades infecciosas, drogas, enfermedades dermatológicas, inmunológicas y/o neoplásicas. Una historia detallada y un examen físico cuidadoso pueden ser esenciales para hacer un diagnóstico correcto. La historia debe incluir el sitio de comienzo, porcentaje y dirección de extensión, presencia o ausencia de prurito y relación temporal con la fiebre. En este artículo se revisarán diagnósticos no infecciosos de exantemas febriles

Stevens-Johnson syndrome due to carbamazepine.

Two patients with psychotic disorders who developed Stevens-Johnson Syndrome while on treatment with carbamazepine is reported due to its rarity. Dermatological side-effects of carbamazepine may be more common in psychiatric as compared to neurological patients.