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1.
Open Med (Wars) ; 19(1): 20240931, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38623456

RESUMO

Aim: This study aimed to summarize case reports of adverse drug reactions (ADRs) caused by piperacillin and explore their effects on human organs in real-world settings. Method: Case reports of piperacillin ADRs were collected by searching databases such as PubMed, Embase, Web of Science, CNKI, WanFang, and VIP from inception to December 2022. Results: A total of 170 patients were ultimately included. The results revealed that ADRs caused by piperacillin were primarily associated with the entire body, followed by the blood system, skin and soft tissues, and the nervous system. The most frequently reported cases included anaphylactic shock, drug fever, rash, and thrombocytopenia. The most severe ADRs were identified as anaphylactic shock and bullous epidermal necrolysis. Furthermore, a comparison was made between systemic adverse reactions caused by piperacillin as a single drug and two composite preparations of piperacillin/ß-lactamase inhibitor. ADRs not mentioned in the instructions included convulsions or hallucinations and Kounis syndrome (KS). Conclusion: This review suggests that the most severe ADRs associated with piperacillin are toxic epidermal necrolysis and anaphylactic shock. Rare ADRs caused by piperacillin, such as myoclonic jerks, hallucinations, and KS, were identified. The most common symptom with domestic preparations of piperacillin/sulbactam and piperacillin sodium was dyspnea.

2.
Am J Emerg Med ; 81: 16-22, 2024 Apr 06.
Artigo em Inglês | MEDLINE | ID: mdl-38631147

RESUMO

INTRODUCTION: Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions that carry a high rate of morbidity and mortality. OBJECTIVE: This review highlights the pearls and pitfalls of SJS/TEN, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: SJS/TEN is a rare, delayed hypersensitivity reaction resulting in de-epithelialization of the skin and mucous membranes. The majority of cases are associated with medication or infection. Clinicians should consider SJS/TEN in any patient presenting with a blistering mucocutaneous eruption. Evaluation of the skin, mucosal, pulmonary, renal, genital, and ocular systems are essential in the diagnosis of SJS/TEN, as well as in the identification of complications (e.g., sepsis). Laboratory and radiological testing cannot confirm the diagnosis in the ED setting, but they may assist in the identification of complications. ED management includes stabilization of airway and breathing, fluid resuscitation, and treatment of any superimposed infections with broad-spectrum antibiotic therapy. All patients with suspected SJS/TEN should be transferred and admitted to a center with burn surgery, critical care, dermatology, and broad specialist availability. CONCLUSIONS: An understanding of SJS/TEN can assist emergency clinicians in diagnosing and managing this potentially deadly disease.

3.
Indian J Dermatol ; 69(1): 24-31, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38572027

RESUMO

Background: Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are life-threatening dermatological emergencies. SCORTEN (SCORe of toxic epidermal necrolysis) is a validated score to predict mortality; however, there is a paucity of data to determine its usefulness in the Indian population. Objective: To evaluate the accuracy of SCORTEN as a prognostic marker in SJS-TEN. Methods: A prospective observational study was conducted at a tertiary care hospital for two years. SCORTEN was calculated on days one and three of admission. The actual death rates were compared to the predicted rates as estimated by the SCORTEN by standardised mortality ratio analysis (SMR). Results: Of 40 cases included in the study, the mean age was 36.2 ± 14 years (range 11-65) with the male: female ratio being 1.67:1. Antibiotics (37.5%) were the most common group followed by anticonvulsants (22.5%). Comorbidities were observed in 60% of cases, with epilepsy (17.5%) and HIV (human immunodeficiency virus) infection (12.5%) being common. On univariate analysis, heart rate > 120/min, epidermal detachment > 10% BSA, and Se HCO3 (bicarbonate) <20 mmol/L were associated significantly with the death of the subjects (P < 0.05). The observed mortalities were 4.34%, 0, 0 and 80% for SCORTEN 0-1 (3.2%), 2 (12.1%), 3 (35.8%) and 4 (58.3%) respectively when compared to expected mortality. SMR of SJS was 0.69 and of TEN was 1.49. Conclusion: SCORTEN gave an overestimation of mortality in patients with lower scores and an underestimation of mortality in patients with higher scores in our study. Minor refinements based on the study population may increase the predictive accuracy of the original scale.

4.
Australas J Dermatol ; 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38572842

RESUMO

BACKGROUND/OBJECTIVES: The immune checkpoint inhibitors (ICIs) have been increasingly associated with severe cutaneous adverse reactions (SCARs). These reactions, including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP) are uncommon but potentially lethal. Despite the severity of these reactions and growing association with the ICIs, their specific risk and mortality rates have been largely unexplored. METHODS: A case/non-case analysis was performed using data from the United States Food and Drug Administration (FDA) Adverse Event Reporting System (FAERS) to examine the reporting odds ratios (RORs) for ICI-associated SCARs cases under two conditions: (1) ICIs compared with all drugs in FAERS and (2) ICIs compared with a reference group of pooled anticancer drugs to control for underlying malignancy. RESULTS: A statistically significant ROR for SJS (ROR: 5.44), TEN (ROR: 5.81) and DRESS (ROR: 1.38) were identified under Condition 1. Under Condition 2, this significance was maintained for SJS (ROR: 7.31), TEN (ROR: 7.40) and DRESS (ROR: 3.90), and mild significance was identified for AGEP (ROR: 1.89). Mortality rates for the ICIs were increased compared with the anticancer medications (28.5% vs. 24.5% for SJS, 55.3% vs. 46% for TEN, 3.0% vs. 2.1% for AGEP and 7.1% vs. 6.1% for DRESS). CONCLUSIONS: Our results suggest an association between SCARs and the ICIs independent of cancer status.

5.
Cureus ; 16(3): e55350, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38559535

RESUMO

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are immune-mediated skin reactions with high mortality as a result of severely compromised skin barrier function. Currently, there is no consensus on the topical management of these conditions. Some advocate for surgical debridement of affected skin as a means of preventing infection and facilitating reepithelialization with synthetic and biological wound coverage. Others prefer a conservative approach that relies on leaving the blistered skin in situ. A consensus is lacking, primarily due to the rarity of the disease and the lack of high-quality evidence supporting one particular form of management. The goal of this review is to explore and compare the two treatment approaches for SJS and TEN, namely conservative management and surgical debridement.

6.
Magy Seb ; 77(1): 28-32, 2024 Apr 02.
Artigo em Húngaro | MEDLINE | ID: mdl-38564285

RESUMO

Bemutatásra kerülo esetünkben egy 47 éves, generalizált septicus állapotú férfi beteg komplex terápiás megoldást igénylo kezelését ismertetjük, negatív nyomásterápia segítségével (NPWT). A páciens kezeletlen diabéteszes láb szindróma talaján kialakult szepszis, fasciitis necrotisans klinikai-radiomorfológiai képével került osztályunkra, akinél sürgosséggel feltárást, az alsó végtag valamennyi kompartmentjét érinto fasciotomiát végeztünk, NPWT-kezelést indítottunk. Kezelése során a beteg állapotát súlyosbító szövodmények léptek fel: Curling-fekély, toxicus epidermalis necrolysis (TEN). A fascitis kapcsán kialakult kb. 6% TBSA (total body surface area) kiterjedésu hámhiányt a TEN-szindróma további epidermális állományvesztéssel tovább súlyosbította. Állapotstabilizálást, kezdeti lokalis kontroll biztosítását követoen a hámhiányos felület csökkentése érdekében a sebeket szukítettük, a feltisztult sebalapok fedése 1:3 arányban hálósított félvastag bor transzplantációjával történt. Az NPWT-kezelést a transzplantációt követoen is folytattuk. A beteg három hónapos intenzív osztályos és sebészeti kezelést követoen sebészi szempontból meggyógyult. A negatív nyomásterápia korai - a kórlefolyásnak megfelelo - adekvát üzemmódban és fedési technikával történo alkalmazása a végtagvesztéssel és életveszéllyel járó nagy fokú hámhiány esetében hatékony eszköznek bizonyult. A multidiszciplináris terápiának köszönhetoen betegünk sebészeti alapbetegségét sikeresen gyógyítottuk, azonban az évtizedes tartamú kezeletlen cukorbetegsége, SARS-Covid peumoniája, a relabáló septicus állapota során fellépo szövodmények következtében felépülni már nem tudott.


Assuntos
Fasciite , Tratamento de Ferimentos com Pressão Negativa , Humanos , Superfície Corporal , Vesículas Transportadoras
7.
J Dermatol ; 2024 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-38605474

RESUMO

Primary cutaneous aspergillosis (PCA) is a rare opportunistic infection caused by Aspergillus that can be life-threatening. PCA is mainly reported in immunocompromised hosts such as patients with AIDS, those with hematologic malignancy, or infants with occlusive dressings. However, no study has previously reported PCA associated with toxic epidermal necrolysis (TEN). This study reports four cases of TEN complicated with PCA, presenting with discrete gray or black spots over newly formed epithelia. Risk factors of PCA in patients with TEN include host factors, iatrogenic factors, indoor environment, and wound care. Two of the four cases eventually died, highlighting the importance of further exploring PCA in patients with TEN.

8.
Cureus ; 16(3): e55318, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38562325

RESUMO

Cutaneous adverse drug reactions (CADRs) are one of the most broadly studied and rigorously researched conditions in recent dermatological advancements. Also termed as "toxidermia," they are heavily involved and are of utmost importance to be understood and studied in the modern healthcare industry. In simple terms, they are dermatological manifestations which result from systemic drug administration to patients. Since allopathy is influenced by the medicines and drugs provided to the patients, cutaneous skin eruptions are a common occurrence in recent times. It is a need of the hour to understand the causative factors for such skin eruptions and the correct management and handling of such disorders to provide better healthcare to patients. The withdrawal of the causative drug which induces the reaction plays a key role in treatment. The risk factors are to be thoroughly studied, and dosages must be in accordance with the patient's situation. They are some of the common public health problems. The age group which is affected is highly variable as people from all age groups can be affected. Those who are affected comprise approximately 10% of all hospitalized patients, and it is also observed in about 1-4% of people who are on multiple medications.

9.
J Inflamm Res ; 17: 2337-2351, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38645875

RESUMO

Objective: Anticancer drugs have revolutionized tumor therapy, with cutaneous toxicities such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) being common immune-related adverse events. The debate over the efficacy of systemic corticosteroids in treating these conditions persists, while tumor necrosis factor (TNF)-alpha inhibitors show promise. This study aims to evaluate the effectiveness and safety of combination therapy involving the TNF-α inhibitor adalimumab for SJS/TEN induced by anticancer drugs. Methods: A literature review of SJS/TEN cases induced by anticancer drugs from 1992 to 2023 was conducted, alongside an analysis of patients admitted to the First Affiliated Hospital of Fujian Medical University during the same period. Clinical characteristics, skin healing time, mortality, and adverse events were evaluated in two treatment groups: SJS/TEN patients treated with targeted anticancer therapies and immunotherapies. Results: Among the 27 patients studied (18 with SJS or SJS-TEN overlapping and 9 with TEN), combination therapy with adalimumab significantly reduced mucocutaneous reepithelization time and healing duration compared to corticosteroid monotherapy. Patients receiving adalimumab combined with corticosteroids had lower actual mortality rates than those on corticosteroid monotherapy. The combination therapy also showed a trend towards reducing standardized mortality rates based on the Score of Toxic Epidermal Necrolysis (SCORTEN). Conclusion: The findings suggest that adalimumab in combination with corticosteroids provides significant clinical benefits and is safer than corticosteroids alone for treating SJS/TEN induced by targeted anticancer therapies and immunotherapies. This study contributes valuable insights into potential treatment strategies for severe cutaneous adverse reactions to anticancer drugs, highlighting the importance of exploring alternative therapies such as TNF-α inhibitors in managing these conditions effectively.

10.
World J Clin Cases ; 12(7): 1371-1377, 2024 Mar 06.
Artigo em Inglês | MEDLINE | ID: mdl-38524512

RESUMO

BACKGROUND: Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are very serious skin allergies, with an etiology related to infections and medication. Since the coronavirus disease 2019 (COVID-19) pandemic, severe acute respiratory syndrome coronavirus-2 has also been considered to cause SJS/TEN. CASE SUMMARY: We report the case of a woman in her thirties who took acetaminophen after contracting COVID-19. After 3 d of fever relief, she experienced high fever and presented with SJS/TEN symptoms, accompanied by intrahepatic cholestasis. Three days of corticosteroid treatment did not alleviate the skin damage; therefore, double plasma molecular adsorption system (DPMAS) therapy was initiated, with treatment intervals of 48 h. Her skin symptoms improved gradually and were resolved after seven DPMAS treatments. CONCLUSION: DPMAS therapy is beneficial for abrogating SJS/TEN because plasma adsorption and perfusion techniques reduce the inflammatory mediators (e.g., tumor necrosis factor-alpha and interleukin-10 and-12) speculated to be involved in the pathology of the skin conditions.

11.
World J Clin Cases ; 12(7): 1290-1295, 2024 Mar 06.
Artigo em Inglês | MEDLINE | ID: mdl-38524518

RESUMO

BACKGROUND: Toxic epidermal necrolysis (TEN) is a life-threatening dermatological emergency mainly induced by drug hypersensitivity reactions. Standard management includes discontinuation of culprit drug and application of immunomodulatory therapy. However, mortality remains high due to complications like septic shock and multiorgan failures. Innovative approaches for skin care are crucial. This report introduces borneol-gypsum, a traditional Chinese drug but a novel dressing serving as an adjuvant of TEN therapy, might significantly improve skin conditions and patient outcomes in TEN. CASE SUMMARY: A 38-year-old woman diagnosed with eosinophilic granulomatosis with polyangiitis experienced gangrenous complications and motor nerve involvement. After initial treatment of high-dose corticosteroids and cyclophosphamide, symptom of foot drop improved, absolute eosinophil counts decreased, while limb pain sustained. Duloxetine was added to alleviate her symptom. Subsequently, TEN developed. Additional topical application of borneol-gypsum dressing not only protected the skin lesions from infection but also significantly eased localized pain. This approach demonstrated its merit in TEN management by promoting skin healing and potentially reducing infection risks. CONCLUSION: Borneol-gypsum dressing is a promising adjuvant that could significantly improve TEN management, skin regeneration, and patient comfort.

12.
JAAD Int ; 15: 139-146, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38545494

RESUMO

Background: Severe cutaneous adverse reactions (SCARs) are associated with morbidity and mortality. Objective: The aim was to determine the different types of SCARs, their morphology, common offending drugs, interventions, and outcomes. Methods: A retrospective cohort study was conducted of all patients admitted to the dermatology service at the University Hospital of the West Indies with Stevens-Johnson syndrome (SJS), SJS/toxic epidermal necrolysis overlap (TEN), TEN, drug reaction with eosinophilia and systemic symptoms and acute generalized exanthematous pustulosis between January 1, 2012 to June 1, 2022. Results: Fifty-one cases (51) met the inclusion criteria for SCAR. SJS, SJS/TEN overlap and TEN together accounted for 71.2% of cases. SCARs were most frequent in the fourth, fifth and 6th decades of life and there was a female preponderance. Antibiotics (31%) and anticonvulsants (29%) were the most common causative agents for SCARs. Most patients had at least 1 complication. The liver was the most common extracutaneous organ affected. Mortality was 7.8%. The main cause of death was sepsis. Limitations: Results were not generalizable. There were missing data and loss to follow-up. Conclusion: Judicious use of antimicrobials and corticosteroids may be beneficial in treatment of severe cutaneous drug reactions.

14.
Pediatr Allergy Immunol Pulmonol ; 37(1): 22-32, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38484271

RESUMO

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare potentially life-threatening hypersensitivity disorders characterized by widespread skin and mucosal involvement. However, there is no standardized evidence-based treatment to reduce the complications of SJS/TEN. This article aims to compare the efficacy of different treatments for pediatric SJS/TEN in terms of length of hospital stay (LOS) using a Bayesian network meta-analysis (NMA). A Bayesian NMA is used to compare and combine evidence from multiple studies and allows clinicians to estimate the relative effectiveness of different treatments/interventions while accounting for heterogeneity in the available evidence. Methods: We conducted a comprehensive electronic database search for studies compatible with our inclusion criteria. Six studies with 103 patients were included in the NMA; of them, 37 patients were treated with intravenous immunoglobulin (IVIG), 37 with systemic corticosteroids (CS), 23 with IVIG + CS, and 3 with Etanercept (ET) + CS. Patients with a median age of 10 years were included in the study. Results: CS had the highest probability of being the most optimal treatment for SJS/TEN in terms of shorter LOS based on the Surface Under the Cumulative Ranking curve levels, and CS + IVIG was associated with a statistically nonsignificant trend toward shorter LOS than IVIG alone. Remarkably, none of the treatments showed a significant benefit over the other interventions in terms of LOS. Conclusion: Current evidence suggests that coadministration of CS and IVIG may be associated with a shorter LOS than IVIG alone. Further research with larger randomized controlled trials is needed to reach a definitive conclusion about the efficacy of specific therapy on LOS in pediatric SJS/TEN and to establish more definitive treatment guidelines.


Assuntos
Síndrome de Stevens-Johnson , Humanos , Criança , Síndrome de Stevens-Johnson/tratamento farmacológico , Síndrome de Stevens-Johnson/etiologia , Imunoglobulinas Intravenosas/uso terapêutico , Tempo de Internação , Teorema de Bayes , Metanálise em Rede , Corticosteroides/uso terapêutico
15.
Hong Kong Med J ; 2024 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-38531617

RESUMO

INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) [hereafter, SJS/TEN] are uncommon but severe mucocutaneous reactions. Although they have been described in many populations worldwide, data from Hong Kong are limited. Here, we explored the epidemiology, disease characteristics, aetiology, morbidity, and mortality of SJS/TEN in Hong Kong. METHODS: This retrospective cohort study included all hospitalised patients who had been diagnosed with SJS/TEN in Prince of Wales Hospital from 1 January 2004 to 31 December 2020. RESULTS: There were 125 cases of SJS/TEN during the 17-year study period. The annual incidence was 5.07 cases per million. The mean age at onset was 51.4 years. The mean maximal body surface area of epidermal detachment was 23%. Overall, patients in 32% of cases required burns unit or intensive care unit admission. Half of the cases involved concomitant sepsis, and 23.2% of cases resulted in multiorgan failure or disseminated intravascular coagulation. The mean length of stay was 23.9 days. The cause of SJS/TEN was attributed to a drug in 91.9% of cases, including 84.2% that involved anticonvulsants, allopurinol, antibiotics, or analgesics. In most cases, patients received treatment comprising either best supportive care alone (35.2%) or combined with intravenous immunoglobulin (43.2%). The in-hospital mortality rate was 21.6%. Major causes of death were multiorgan failure and/or fulminant sepsis (81.5%). CONCLUSION: This study showed that SJS/TEN are uncommon in Hong Kong but can cause substantial morbidity and mortality. Early recognition, prompt withdrawal of offending agents, and multidisciplinary supportive management are essential for improving clinical outcomes.

16.
Ophthalmol Ther ; 2024 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-38507192

RESUMO

INTRODUCTION: This study analysed the causative factors and clinical characteristics of acute and chronic ocular sequelae of Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) treated at a large third-referral centre in a developed country (Hungary) over a 15-year period. METHODS: This was a retrospective review of patients with acute and/or chronic SJS/TEN who were managed between 2006 and 2020 at the Department of Ophthalmology of Semmelweis University in Budapest, Hungary. For each subject, clinical data, including patient demographics, clinical history, causative agents of SJS/TEN, and conservative and surgical treatment details, were reviewed. RESULTS: Ninety-six eyes of 48 patients were included (28 female; 58.3%); the age at disease onset was 32.1 ± 22.4 years. The most common causative factors were medicines (n = 36; 75.0%). Among these drugs, 29.2% were nonsteroidal anti-inflammatory drugs (NSAIDs) (n = 14), 20.8% were antibiotics (n = 10) and 14.6% were antiepileptic drugs (n = 7). In patients with chronic SJS/TEN, the most commonly found ocular sequelae were conjunctival hyperaemia in 45 (56.3%) eyes, symblepharon in 38 (47.5%) eyes, trichiasis/distichiasis in 37 (46.3%) eyes, corneal neovascularization in 31 (38.8%) eyes and corneal scarring in 29 (36.3%) eyes. In patients with chronic SJS/TEN, the most frequently used topical conservative treatment included antibiotics in 53 (66.3%) eyes, preservative-free artificial tears in 50 (62.5%) eyes and topical corticosteroids in 42 (52.5%) eyes of 40 patients. The most frequently performed ocular surgeries for managing chronic ocular sequelae in patients with SJS/TEN were epilation for trichiasis (n = 27; 33.8%), cataract surgery (n = 14; 17.5%), entropion surgery (n = 12; 15.0%), penetrating keratoplasty (PK) (n = 11; 13.8%) and amniotic membrane transplantation (n = 4; 5.0%). CONCLUSION: Our results suggest that NSAIDs, antibiotics and antiepileptic drugs are the most common causative factors for SJS/TEN in Hungary. Like in other countries, in Hungary, the ocular management of patients with acute and chronic SJS/TEN is heterogeneous, and most cases do not follow modern therapeutic guidelines.

17.
Proc (Bayl Univ Med Cent) ; 37(2): 336-338, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38343491

RESUMO

Stevens-Johnson syndrome and toxic epidermal necrolysis are potentially life-threatening cutaneous diseases that are rarely seen in the pediatric population. We describe a case of a 7-year-old female who presented with fever, cough, mucosal erosions, and a widespread maculopapular eruption with flaccid bullae for 4 days. She had no sick contacts and no new medications, supplements, or vitamins. She then rapidly developed ulcerations in her oral mucosa and genital area, numerous scattered tense bullae throughout her body, and patchy areas of desquamation. Infectious disease workup revealed positivity only for Bordetella parapertussis, and she was subsequently diagnosed with idiopathic toxic epidermal necrolysis.

18.
Photodiagnosis Photodyn Ther ; 46: 104011, 2024 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-38360083

RESUMO

Characterized by an immune reaction to medications, toxic epidermal necrolysis (TEN) and Steven-Johnson Syndrome (SJS) are potentially fatal mucocutaneous reactions, and their management remains challenging. Considering the promising studies regarding the use of laser in managing orofacial lesions, this study aimed to report two cases in which children presenting with TEN and SJS were treated using a combination of antimicrobial photodynamic therapy (aPDT) and photobiomodulation therapy (PBMT) concurrently with conventional supportive care. The treatment proposed herein resulted in significant clinical improvement of the children's oral condition within a few days, enabling the reintroduction of oral feeding. Within the limitations of the study, the cases reported suggest that the adjuvant combination of PBMT and aPDT may be beneficial for improving the oral condition of children afflicted with oral injuries induced by TEN and SJS.

19.
Cureus ; 16(1): e52222, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38347997

RESUMO

Toxic epidermal necrolysis (TEN) is a rare and life-threatening cutaneous disease, frequently triggered by drugs. Allopurinol is one of the most frequent drugs associated with TEN, which implies detachment of a significant amount of the body surface area (BSA) and has a high morbidity and mortality associated with it. We present the case of a 68-year-old female with a recent diagnosis of hyperuricemia who started treatment with allopurinol. A week later, she presented to the emergency department with an extensive maculopapular exanthema with blisters and skin detachment. After the exclusion of other etiologies, the diagnosis of allopurinol-induced TEN was made, with 35% of BSA involvement. Due to the severity of the clinical condition, she was admitted to intensive care and treated with corticoids that had no response. So, she was started on immunoglobulins and transferred to a burn unit. She developed sepsis with multiorgan failure and required supportive treatment. She was discharged after a month, and physical rehabilitation was needed. This clinical case highlights the severity of allopurinol hypersensitivity that may happen and the importance of an accurate diagnosis and treatment for this rare disease.

20.
Front Immunol ; 15: 1342898, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38333208

RESUMO

Toxic epidermal necrolysis (TEN) is a life-threatening mucocutaneous disorder commonly caused by drugs. TEN is often treated with corticosteroids, intravenous immunoglobulin (IVIG), or cyclosporine; however, the efficacy of these treatments is controversial. Etanercept (a TNF-α antagonist) was proven to decrease skin-healing time in a randomized clinical trial. Herein, we report the case of a 44-month-old boy who developed TEN due to deflazacort as the probable culprit drug and was successfully treated with etanercept. The patient presented to the emergency department complaining of erythematous maculopapular rashes and vesicles all over the face and body, with vesicles on the hands, feet, and trunk. Symptoms started 4 days before presentation, with edema of the upper lip, which progressed to erythematous macules over the body. He was started on deflazacort for nephrotic syndrome 21 days before the visit. Approximately 20% of the body surface area (BSA) was covered by vesicular lesions. Under the diagnosis of Steven Johnson syndrome/TEN, deflazacort was discontinued, and intravenous dexamethasone (1.5 mg/kg/day), a 5-day course of IVIG (0.4 mg/kg/day), and cyclosporine (3 mg/kg/day) were administered. The lesions seemed to be stationary for 3 days, but on the 6th day of hospitalization, when IVIG was discontinued, the vesicular lesions progressed to approximately 60% of the BSA. Etanercept 0.8 mg/kg was administered subcutaneously. Lesions stopped progressing, and bullous lesions started epithelialization. However, on the 15th day, around 30% of the BSA was still involved; thus, a second dose of etanercept was administered. No acute or sub-acute complications were observed. In conclusion, the use of etanercept in children with TEN that is not controlled with conventional therapy is both effective and safe.


Assuntos
Etanercepte , Síndrome de Stevens-Johnson , Pré-Escolar , Humanos , Masculino , Etanercepte/uso terapêutico , Pregnenodionas/toxicidade , Ensaios Clínicos Controlados Aleatórios como Assunto , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/tratamento farmacológico , Síndrome de Stevens-Johnson/etiologia
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