ACETAMINOPHEN-INDUCED TOXIC EPIDERMAL NECROLYSIS IN PEDIATRIC PATIENTS-A CASE SERIES

Acetaminophen is the most widely used over-the-counter medication for treating fever and pain. While adverse reactions to this drug are infrequent, they can occasionally result in severe and potentially fatal events, such as Toxic Epidermal Necrolysis (TEN). Due to the rarity of such reactions, there is a limited amount of information available about toxic epidermal necrolysis caused by acetaminophen. This case series will contribute to the existing knowledge in this area. In our cases, acetaminophen is the most suspected drug for the development of toxic epidermal necrolysis in patients. Causality assessment in all of these adverse drug reaction in context with World Health Organization (WHO) causality assessment scale suggests “Possible.” This case series concludes that severe hypersensitivity reactions like TEN caused by acetaminophen use and which can be potentially life-threatening which needs additional treatment.

Amikacin induced Stevens Johnson syndrome and toxic epidermal necrolysis overlap: a rare case report

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the most severe adverse drug reactions. They are characterized by necrosis and epidermal release in vesicobulosa skin, mucous orifice, and eyes, with more severe general symptoms. SJS/TEN-overlap syndrome is the term used to characterize situations where 10–30% of the body skin area is detached. TEN or SJS is one of the deadliest dermatological catastrophes. Despite being a rare condition, it frequently has a high death rate. Different types of purpuric macules or rounded patches with mucosal lesions are characteristic features of these type 3 hypersensitivity reactions. We are presenting a case of 50-year-old female patient brought to hospital on account of multiple black to red coloured raised lesions over body in the last 8 days. Patient was asymptomatic in the last 8 days after that patient noted red coloured lesions first over face followed by lower back, upper limb, lower limb followed by itching and Jiburning sensation all over the body. The patient had symptoms such as redness of eyes in the last 3-4 days, burning sensation and ulcers in mouth in the last 3-4 days. These symptoms developed day after she received amikacin. She was diagnosed with SJS-TEN overlap. This case highlights the precipitant of antibiotics for SJS. She was recovered completely after stopping the causative drug and treatment with Immunoglobulin with other symptomatic measures. The causative drug was found to be Amikacin and medicine taken for comorbid conditions like epilepsy, hypothyroidism and hypertension. Rarely, amikacin has been linked to a range of drug hypersensitivity reactions, including toxic epidermal necrolysis, Stevens-Johnson syndrome, and overlap between the two conditions.

Necrólisis epidérmica tóxica debido a un fármaco a base de caléndula más acetilcisteína: presentación de caso / Toxic Epidermal Necrolysis Induced by a Drug Combination of Calendula and Acetylcysteine: A Case Report / Necrólise epidermal tóxica por medicamento à base de calêndula mais acetilcisteína: relato de caso

Introducción: la hipersensibilidad por fármacos es inducida por una respuesta aberrante del sistema inmune; por lo general, impredecible, dosis independiente y amenazante para la vida del paciente. Las reacciones de hipersensibilidad por fármacos con compromiso mucocutáneo se dan en alrededor del 2 %-3 % de los pacientes hospitalizados. Objetivo: describir un caso de necrólisis tóxica epidérmica por un fármaco a base de caléndula y acetilcisteína como los presuntos desencadenantes. Presentación del caso: hombre de 39 años, quien asistió a emergencias, por malestar general, artralgias, mialgias, astenia y adinamia de un día de evolución. Tres días después refirió la aparición de lesiones purpúricas progre-sivas que se extendieron por cara, extremidades y glúteos; delimitadas; no dolorosas, y sin desaparecer a la digitopresión, asociado con disnea, inestabilidad hemodinámica y cardiovascular. Al sexto día de hos-pitalización, se interrogó al paciente, quien se había automedicado un fármaco de origen desconocido, presuntamente de extracto herbal a base de caléndula más acetilcisteína para artralgias, osteomialgias y disfagia. A la mañana siguiente, presentó lesiones purpúricas y ulcerativas extensas. Se observaron lesiones purpúricas progresivas y ulcerativas en cara extremidades y glúteos. Mediante la sospecha clínica y la evaluación histopatológica, se confirmó la necrólisis tóxica epidérmica. Conclusiones: las reacciones adversas medicamentosas severas que amenazan la vida del paciente son relativamente raras, pero representan un verdadero desafío diagnóstico y terapéutico. Es necesario profundizar en la investigación para esclarecer la causa de este tipo de reacción medicamentosa.

Introduction: Drug hypersensitivity is induced by an aberrant response from the immune system. It is usually unpredictable, dose-independent, and can be life-threatening to the patient. Drug-induced hypersensitivity reactions with mucocutaneous involvement occur in approximately 2­3% of hospitalized patients. However, Toxic Epidermal Necrolysis is a rare case. Aim: To describe a case of drug-induced toxic epidermal necrolysis triggered by Calendula officinalis and acetylcysteine. Case report: A 39-year-old male was pre-sented to the emergency room with malaise, arthralgia, myalgia, asthenia, and adynamia since day one. Three days later, he developed progressive purpuric lesions that spread to the face, extremities, and buttocks. These lesions were painless, not delineate, and did not blanch under pressure. They were asso-ciated with dyspnea and hemodynamic and cardiovascular instability. On the sixth day of hospitaliza-tion, the patient admitted to self-medicating with an unknown drug, presumably a herbal extract based on calendula and acetylcysteine, for arthralgia, myalgia, and dysphagia. The morning after the drug consumption, the patient developed extensive purpuric and ulcerative lesions. A diagnosis of toxic epi-dermal necrolysis was made based on clinical suspicion and histopathological confirmation. Conclusion:Severe adverse drug reactions that threaten a patient's life are relatively rare. However, they represent a real diagnostic and therapeutic challenge when they do occur

Introdução: a hipersensibilidade a drogas é induzida por uma resposta aberrante do sistema imunoló-gico; geralmente imprevisível, independente da dose e com risco de vida para o paciente. As reações de hipersensibilidade a drogas com comprometimento mucocutâneo estão em torno dos 2-3% dos pacien-tes hospitalizados. Objetivo: descrever um caso de necrólise epidérmica tóxica causada por uma droga à base de Calendula officinalis e acetilcisteína como os supostos desencadeantes. Apresentação do caso:homem, 39 anos, deu entrada no pronto-socorro manifestando mal-estar, artralgia, mialgia, astenia e adinamia há um dia. Posteriormente, três dias depois, relatou o aparecimento de lesões purpúricas pro-gressivas que se espalharam para a face, extremidades e nádegas; que são delimitadas, não dolorosas e não desaparecem com a acupressão associada a dispnéia, instabilidade hemodinâmica e cardiovascular. No sexto dia de internação, o paciente foi questionado, y disse que tinha se automedicado com uma droga de origem desconhecida, presumivelmente extrato de ervas à base de calêndula mais acetilcis-teína para artralgia, osteomialgia e disfagia. Na manhã seguinte, apresentou extensas lesões purpúricas e ulcerativas. Observam-se lesões purpúricas progressivas e ulcerativas que se estendem à face, extre-midades e nádegas. Pela suspeita clínica e confirmação histopatológica, confirma-se a necrólise epidér-mica tóxica. Conclusões: reações adversas graves a medicamentos que ameaçam a vida do paciente são relativamente raras, mas quando ocorrem representam um verdadeiro desafio a nível diagnóstico e terapêutico. São precisas mais pesquisas para esclarecer a causa desse tipo de reação medicamentosa.

A case of toxic epidermal necrolysis caused by sorafenib and sintilimab / 药物流行病学杂志

A 67-year-old female patient with postoperative recurrence of stage Ⅳright renal cell carcinoma and multiple intracranial metastases was treated with sorafenib and sintilimab.Within 2 weeks,the patient had a fever and red spotted rash in facial,back,buttocks and limb.After 2 days,the fever completely relieved,but subcutaneous exudation appeared on the skin of both elbow joints,buttocks,and outer thighs,followed by gradual epidermal lysis and detachment with skin ulceration.After 4 days,the patient's epidermolysis area was greater than 30％of the body surface area.The patient was diagnosed with toxic epidermal necrolysis(TEN).The adverse reaction correlation was assessed by ALDEN SCORE sheet.The adverse reaction of TEN was"likely"caused by sorafenib and sintilimab.After withdrawal and treatment,the TEN was cured.This paper explores the correlation between the TEN and the combination use of sorafenib and sintilimab and the management.This paper will provide reference for the early diagnosis and correct treatment of TEN.

Progress in plasma exchange for severe immune-related dermatoses / 中国输血杂志

Plasma exchange (PE) is effective in a part of autoimmune diseases, and its main action mechanisms include removing pathogenic factors from the patient's blood, regulating immune function, replenishing normal plasma components. The use of PE in the treatment of severe immune-related skin diseases, such as pemphigus vulgaris, toxic epidermal necrolysis and clinically amyopathic dermatomyositis, has become more widespread. This review provides an overview of the progress of PE application in severe immune-related skin diseases at home and abroad in recent years to provide new ideas for the treatment of clinical severe skin diseases.

Síndrome Stevens Jonhson: reporte de caso clínico / Stevens Johnson syndrome: clinical case report

Resumen Introducción: El síndrome Stevens Johnson (SSJ) es una dermatosis potencialmente fatal caracterizada por una extensa necrosis epidérmica y de mucosas que se acompaña de ataque al estado general, y junto con la necrólisis epidérmica tóxica (NET) se consideran reacciones de hipersensibilidad tipo IV, relacionadas con ciertos fármacos en 60% de los casos, siendo uno de los diagnósticos pocos frecuentes, pero con una alta mortalidad hasta del 40%. Caso clínico: El siguiente caso clínico es un masculino de 34 años de edad que inició un cuadro de eritema generalizado inmediatamente tras la administración del medicamento trimetoprima/sulfametoxazol. Se le solicitó un hemograma mostrando leucocitosis, neutrofilia, VSG elevada, PCR elevada, IgE elevada, y tras el interrogatorio clínico se realiza el algoritmo ALDEN dando positivo con 10 puntos asociado al medicamento previamente dicho. Por lo tanto se le inicia tratamiento con metilprednisolona, difenhidramina, inmunoglobulina humana intravenosa y un plan terapéutico cutáneo, dando como resultado una mejoría clínica, evitando complicaciones y secuelas, hasta el día de su egreso. A manera de conclusión, se requiere un manejo multidisciplinario para atender las manifestaciones clínicas del inmunoglobulina humana intravenosa.

Abstract Introduction: Stevens Johnson Syndrome (SJS) is a potentially fatal dermatosis characterized by extensive epidermal and mucosal necrosis accompanied by an attack on the general condition, which together with Toxic Epidermal Necrolysis (TEN) are considered type IV hypersensitivity reactions, related to certain drugs in 60% of cases, being one of the rare diagnoses, but with a high mortality of up to 40%. Case report: The following clinical case is a 34 year old male who started a generalized erythema picture immediately after administration of the medication trimethoprim/sulfamethoxazole, for which a complete blood count was requested showing leukocytosis, neutrophilia, elevated ESR, elevated PCR, elevated IgE, and after the clinical questioning, the ALDEN algorithm was performed, giving positive with 10 points associated with the previously mentioned medication, for which treatment was started with methylprednisolone, diphenhydramine, intravenous human immunoglobulin and a skin therapeutic plan, resulting in clinical improvement, avoiding complications and sequelae, until the day of discharge. In conclusion, a multidisciplinary management is required to attend to the clinical manifestations of the patient, helping him to a quick and effective recovery.

Síndrome de Stevens-Johnson y Necrólisis epidérmica tóxica: presentación en un paciente pediátrico / Stevens-Johnson syndrome and toxic epidermal necrolysis: presentation in a pediatric patient

El síndrome de Stevens-Johnson (SSJ) y la necrólisis epidérmica tóxica (NET), constituyen el espectro de una enfermedad aguda, originada por una reacción de hipersensibilidad, secundaria a ingesta de medicamentos o infecciones, que afecta la piel y las membranas mucosas, causadas por apoptosis y posterior necrosis de los queratinocitos. Se presenta un escolar masculino de 8 años de edad, con antecedente de epilepsia estructural, a quien en su último control por Neuropediatría, se le indicó tratamiento con Lamotrigina, presentando posteriormente lesiones tipo pápulas faciales, que progresaron rápidamente en sentido céfalo caudal; a las 48 horas, las lesiones evolucionaron a pústulas en mentón, y posteriormente a flictenas. Se utilizaron medidas de soporte vital, limpieza quirúrgica, obteniéndose mejoría clínica progresiva, incluyendo recuperación de las lesiones en piel. El aislamiento temprano por contacto, los cuidados de la piel y la mínima invasión, fueron factores fundamentales en la evolución satisfactoria de este paciente(AU)

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) constitute the spectrum of an acute disease, caused by a hypersensitivity reaction, secondary to medication intake or infections, which affects the skin and mucous membranes. Caused by apoptosis and subsequent necrosis of keratinocytes. An 8-year-old male schoolboy is presented, with a history of structural epilepsy, who was prescribed treatment with Lamotrigine during his last Neuropediatric check-up, subsequently presenting facial papule-type lesions, which progressed rapidly in the cephalocaudal direction; 48 hours later, the lesions evolved into pustules on the chin, and later to blisters. Life support measures and surgical cleaning wereused, obtaining progressive clinical improvement, including recovery of skin lesions. Early contact isolation, skin care and minimal invasion were fundamental factors in the satisfactory evolution of this patient(AU)

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.

Múltiples reacciones adversas graves a fármacos en un paciente luego de la infección por COVID-19: informe de un caso / Drug-induced severe cutaneous adverse reactions in a patient after COVID-19 infection: case report

Durante la pandemia por COVID-19 se observaron diversas reacciones adversas a fármacos. Esto pudo haber estado relacionado con una mayor susceptibilidad inmunológica de los pacientes con SARS-CoV-2 a presentar este tipo de cuadros, así como también con la exposición a múltiples medicamentos utilizados en su tratamiento. Comunicamos el caso de un paciente con una infección respiratoria grave por COVID-19, que presentó 2 reacciones adversas graves a fármacos en un período corto de tiempo. (AU)

During the COVID-19 pandemic, various adverse drug reactions were observed. This could have been related to a greater immunological susceptibility of patients with SARS-CoV-2 to present this type of symptoms, as well as exposure to multiple drugs used in their treatment. We report the case of a patient with a severe respiratory infection due to COVID-19, who presented 2 serious adverse drug reactions associated with paracetamol in a short period of time. (AU)

A case report on ofloxacin-induced Stevens–Johnson syndrome: Scorten-based approach

Stevens–Johnson syndrome (SJS) is a rare immune-mediated severe cutaneous adverse reaction with an incidence rate of 0.05–2 persons/million population/month. Drugs are the most commonly implicated in 95% of cases. In our report, a 52-year-old male patient presented with chief complaints of skin rashes over the body and was having a history of using a tab. ofloxacin for gastroenteritis. The severity of SJS was assessed using SCORTEN (=1). The drug can be considered as a probable/likely cause of adverse drug reaction as per causality assessment of the suspected adverse drug reactions. Early diagnosis helps the clinician to elude secondary infection and subsequent complications. It highlights the mandatory reporting of the offending drug and the necessity of pharmacovigilance in different countries.

Clinical study on toxic epidermal necrolysis in 11 children / 中国小儿急救医学

Objective:To investigate the clinical characteristics and treatment strategies of toxic epidermal necrolysis(TEN) in children.Methods:The clinical data, laboratory examination, diagnosis and treatment process and treatment outcomes of 11 children with TEN treated in PICU at Children′s Hospital of Xi′an Jiaotong University from January 2018 to June 2022 were collected and analyzed retrospectively.Results:There were five males and six females, aged from one year to 11 years old.Nine cases were caused by drugs, among which six cases were caused by antiepileptic drugs, two cases were caused by Chinese patent medicine, one case was caused by antibiotics, and the remaining two cases were caused by infection.In addition to large area of skin exfoliation, one case was complicated with corneal perforation, one case was complicated with finger (toe) nail fall off, and two cases were complicated with upper airway obstruction.All children were complicated with various degrees of heart, liver, kidney and other organ damage.Eight cases who were admitted to the department of immunology were treated with methylprednisolone and intravenous immune globulin.Three cases had no change in rash, and five cases had an enlarged range of skin lesions compared with admission, and finally all of them were transferred to PICU.Three cases were first diagnosed in PICU and were not treated with methylprednisolone and intravenous immune globulin after admission.All 11 children were given therapeutic plasma exchange in PICU.For children whose first department was PICU, the average hospitalization time of PICU was (8.00±3.00) days, the total average hospitalization time was (33.66±20.10) days, and the average hospitalization cost was (73.9±30.5) thousand yuan.For children whose first department was the immunology department, the average hospitalization time of PICU was (21.62±16.18) days, the total average hospitalization time was (41.87±16.97) days, and the average hospitalization cost was (130.8±52.2) thousand yuan.One case, because of corneal perforation, the family members asked to leave the hospital for economic reasons after the rash improved, and the rest of the children were cured and discharged.Conclusion:TEN is rare, often complicated with multiple organ dysfunction, and has a high mortality.Early administration of therapeutic plasma exchange may alleviate multiple system damage and shorten the duration of disease.

Síndrome de stevens johnson, a propósito de un caso / Stevens Johnson syndrome: about a case

El síndrome de Stevens Jonson y la Necrolisis Epidérmica Toxica forman parte de un mismo espectro clínico caracterizado por lesiones cutáneas y mucosas con necrosis y desprendimiento de la piel, se diferencian por la extensión y la gravedad de las lesiones, aunque existen casos traslapados como en este caso, donde la paciente presentaba lesiones en más del 30% del cuerpo pero, solo fueron lesiones cutáneas y no así mucosas, lo que favoreció a su recuperación exitosa sin necesidad de ingresar a un servicio de terapia intensiva.

The Stevens Johnson syndrome and Toxic Epidermal Necrolysis are part of the same clinical spectrum characterized by cutaneous lesions and mucous membranes with necrosis and detachment of the skin. They differ by the extent and severity of the lesions. Although, there are overlapping cases as in this case, where the patient had lesions in more than 30% of the body. But they were only skin lesions and not mucous membranes, which favored her successful recovery without the need to enter and ITS (intensive therapy service).

Uso del lisado plaquetario en el tratamiento de la necrolisis tóxica epidérmica: presentación de un caso / Use of platelet lysate in the treatment of epidermal toxic necrolysis: a case presentation

Introducción: En los últimos años se ha producido un extraordinario impulso de la medicina regenerativa. El uso de las plaquetas con este fin, se ha empleado en diferentes especialidades médicas. El lisado plaquetario es una de las alternativas al tratamiento utilizadas en la necrolisis tóxica epidérmica, urgencia dermatológica que alcanza una mortalidad de hasta el 70 por ciento en ocasiones. Objetivo: Describir el uso del lisado de plaquetas como terapia regenerativa en el tratamiento de la necrolisis tóxica epidérmica. Presentación de caso: Paciente de 53 años con antecedentes patológicos de hipertensión arterial, diabetes mellitus, mieloma múltiple e insuficiencia renal crónica, que ingresó con diagnóstico de neumonía adquirida en la comunidad e insuficiencia renal crónica agudizada, para lo que llevó múltiples tratamientos. Comenzó con lesiones en piel eritematosas, no pruriginosas y después ampollares que se desbridan con facilidad, y que están diseminadas por todo el cuerpo con grandes áreas de piel denudada con una mortalidad de más del 90 por ciento. Se diagnosticó una necrolisis tóxica epidérmica, se iniciaron las medidas habituales de tratamiento y se utilizó de forma tópica el lisado plaquetario alogénico. Conclusiones: La aplicación del lisado plaquetario tuvo una respuesta favorable, se observó reepitelización cutánea y mejoría de las lesiones en la necrolisis tóxica epidérmica(AU)

Introduction: In recent years there has been an extraordinary impulse of regenerative medicine, the use of platelets for this purpose has been used in different medical specialties. The most feared of the dermatological complications is toxic epidermal necrolysis, of which up to 4 cases per million inhabitants occur annually, with a mortality that sometimes reaches up to 70 percent Objective: To describe the use of platelet lysate as regenerative therapy in the treatment of epidermal toxic necrolysis. Case presentation: We present a case of a 53-year-old patient with a pathological history of arterial hypertension, diabetes mellitus, multiple myeloma and chronic renal insufficiency, who was admitted with a diagnosis of community acquired pneumonia and acute chronic renal insufficiency, for which multiple treatments. She began with erythematous, non-pruritic, and then bullous skin lesions that are easily debrided into large areas of denuded skin, scattered throughout the body. A toxic epidermal necrolysis was diagnosed, the usual treatment measures were started and the allogeneic platelet lysate was used topically. Conclusions: A favorable response was observed, with cutaneous re-epithelialization of the skin, although the patient's underlying disease and its comorbidities died(AU)

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.

A Report of Staphylococcus Scalded Skin Syndrome in Adult

Summary@#Staphylococcal scalded skin syndrome (SSSS) is typically a clinical diagnosis,1 affecting primarily neonates and children. It is characterised by a diffuse skin disorder with tenderness, erythema, large wrinkled superficial blistering, and desquamation caused by the hematogenous dissemination of exotoxin-producing strains of staphylococcus aureus to the skin.4,10 Hospital admission is required for intravenous anti-staphylococcal antibiotic therapy and supportive care. The rarity of SSSS in adults is best explained by the presence of exotoxins neutralizing antibodies and renal elimination of the toxins.2 Two major risk factors are kidney failure and immunosuppression. Therefore, SSSS in adults warrants thorough evaluation.3 Mortality is also greater than 60% in adults, attributed to predisposing comorbid conditions.1,4 One of the mimickers of SSSS is toxic epidermal necrolysis (TEN). Here, we report a successful treatment of SSSS in an adult with recreational drug abuse and incidental liver cirrhosis possibly secondary to hepatitis C viral infection, after careful exclusion of TEN.

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.

Reacciones cutáneas adversas graves en la infancia / Severe adverse skin reactions in the childhood

RESUMEN Las reacciones cutáneas a drogas son cada vez más frecuentes en edades pediátricas, con un alto impacto en la salud de los niños. Pueden manifestarse en formas muy disímiles, desde un exantema transitorio hasta cuadros graves con afectación multisistémica potencialmente fatales. En la presente revisión se hace énfasis en las farmacodermias graves en la infancia, con el objetivo de promover el conocimiento por parte del personal médico para facilitar su diagnóstico y tratamiento oportuno. Se desarrolló una búsqueda en la Biblioteca Virtual de Salud de Infomed y en Google: se revisaron 28 trabajos científicos sin limitación de año y país, 24 de ellos pertenecen a los últimos cinco años y de estos 17 a los últimos tres. El dominio de los elementos para el diagnóstico precoz y las opciones terapéuticas son indispensables para elegir la conducta adecuada frente a estas reacciones cutáneas graves y disminuir la morbimortalidad por estas afecciones (AU).

ABSTRACT Skin reactions to drugs are increasingly common at pediatric ages, with a high impact on children's health. They can appear in very dissimilar forms, from a transient rash to serious pictures with potentially fatal multisystem involvement. This review focuses on severe pharmacodermies in the childhood, with the aim of promoting medical staff knowledge to facilitate their timely diagnosis and treatment. A search was led in the Infomed Virtual Health Library and in Google: 28 scientific papers were reviewed without limitation of year and country, 24 of them belong to the last five years and from these 17 to the last three. Mastery of the elements for early diagnosis and therapeutic options are indispensable to choose the appropriate behavior against these serious skin reactions and to decrease morbidity and mortality due to these conditions (AU).

Toxic epidermal necrolysis associated with anticonvulsants / Necrólisis epidérmica tóxica asociada a anticonvulsivantes

ABSTRACT Background and Objectives: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as a continuum of the same process. TEN or Lyell Syndrome is the most severe form. Both entities involve an acute mucocutaneous blistering reaction associated with systemic inflammation. Materials and Methods: We present a case of a young woman who developed TEN following concomitant treatment with valproate, lamotrigine, and phenobarbital. Despite the extensive mucocutaneous detachment (over 90%), prognostic evaluation was favorable (SCORTEN score 2; probability of survival 88%), and this patient evolved satisfactorily. Five days after admission, valproate was reinitiated without any subsequent adverse reaction. Results: Causality evaluation identified both lamotrigine and phenobarbital as "very probable" (ALDEN score = 6) causes and valproate as "very unlikely" (ALDEN score = 0) cause of TEN. Conclusions: SJS and TEN are true life-threatening medical emergencies. This case emphasizes the importance of early diagnosis and treatment, including the discontinuation of the causative agent, which can be lifesaving.

RESUMEN Antecedentes y objetivos: El síndrome de Stevens-Johnson (SSJ) y la necrólisis epidérmica tóxica (NET) se consideran un continuum del mismo proceso. La NET o síndrome de Lyell es la forma más grave. Ambas entidades implican una reacción ampollosa mucocutánea aguda asociada con inflamación sistémica. Materiales y métodos: Presentamos el caso de una mujer joven que desarrolló NET posterior al tratamiento concomitante con valproato, lamotrigina y fenobarbital. A pesar del extenso desprendimiento mucocutáneo (más del 90%), la evaluación pronóstica fue favorable (puntuación SCORTEN 2; probabilidad de supervivencia 88%), y esta paciente evolucionó satisfactoriamente. Cinco días después de su ingreso, se reinició el valproato sin ninguna reacción adversa posterior. Resultados: La evaluación de causalidad identificó tanto la lamotrigina como el fenobarbital como causas "muy probables" (puntuación ALDEN = 6) y el valproato como causas "muy improbables" (puntuación ALDEN = 0) de NET. Conclusiones: El SJS y la NET son verdaderas emergencias médicas potencialmente letales. Este caso enfatiza la importancia del diagnóstico y tratamiento tempranos, incluida la interrupción del agente causal, lo cual puede salvar la vida del paciente.

Enfermedad de injerto contra huésped cutánea frente a necrólisis epidérmica tóxica en un receptor de trasplante de células madre hematopoyéticas / Cutaneous graft-versus-host disease versus toxic epidermal necrolysis in a hematopoietic stem cell transplant recipient

El diagnóstico diferencial entre la enfermedad de injerto contra huésped aguda grave (estadio IV) y la necrólisis epidérmica tóxica pude resultar difícil en el contexto de un paciente trasplantado, ya que ambas tienen presentaciones clínicas similares. Sin embargo, la distinción entre ellas es fundamental porque ocasionan una gran morbimortalidad, y su manejo y pronóstico difieren. Algunas pequeñas diferencias clínicas e histopatológicas son de gran ayuda para el diagnóstico diferencial y el dermatólogo deberá reconocerlas para tomar una conducta correcta y oportuna. Se comunica el caso de un paciente que presentó ampollas y epidermólisis después del trasplante de células hematopoyéticas y en el que se planteó la dificultad diagnóstica para diferenciar entre ambas afecciones.

The differental diagnosis between severe graft-versus-host disease (stage IV) and toxic epidermal necrolysis can be difficult in the context of a transplant patient, since both conditions have similar clinical presentations. However, the distinction between these two entities is critical because they produce great morbidity and mortality and their management and prognosis differ. Some small clinical and histopathological differences are of great help for the differential diagnosis, and the dermatologist must recognize them in order to take a correct and timely conduct. We present the case of a patient who developed blisters and epidermolysis after hematopoietic cell transplantation, and in whom the diagnostic difficulty to differentiate between the two entities was raised.

Drug-induced erythroderma in patients with acquired immunodeficiency syndrome / 世界急诊医学杂志（英文）

@#BACKGROUND: To explore the clinical manifestations, diagnosis, and treatment of patients with acquired immunodeficiency syndrome (AIDS) complicated with drug-induced erythroderma. METHODS: The clinical data of 12 AIDS patients with drug-induced erythroderma in our hospital were retrospectively analyzed. The general information, offending medications, complications, modified severity-of-illness score for toxic epidermal necrolysis (SCORTEN) scores, and disease outcome spectrums were analyzed. RESULTS: Drug-induced erythroderma was mostly caused by antiviral drugs, antituberculosis drugs, antibiotics, traditional Chinese medicine, and immune checkpoint inhibitors. The spectrum of sensitizing drugs was broad, the clinical situation was complex, and infections were common. The affected areas were greater than 40% body surface area in all patients. The modified SCOTERN score averaged 3.01±0.99. All patients were treated with glucocorticoids, and nine patients were treated with intravenous immunoglobulin (IVIG) pulse therapy at the same time. The average time to effectiveness was 7.08±2.23 days, and the average hospital stay was 17.92±8.46 days. Eleven patients were cured, and one patient died of secondary multiple infections, who had a modified SCORTEN score of 5 points. The mortality rate in this study was 8.3%. CONCLUSIONS: The clinical situation of AIDS patients with drug-induced erythroderma in hospitalized patients is complex and the co-infection rate is high. The use of modified SCORTEN score may objectively and accurately assess the conditions, and the use of glucocorticoid combined with IVIG therapy may improve the prognosis.