Assuntos
Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/terapia , Síndrome de Stevens-Johnson/etiologia , Adolescente , Lamotrigina/uso terapêutico , Lamotrigina/efeitos adversos , Anticonvulsivantes/uso terapêutico , Anticonvulsivantes/efeitos adversos , Masculino , Acuidade VisualRESUMO
Stevens-Johnson syndrome is an infrequent condition affecting the skin and mucous membranes, it involves cutaneous detachment with high mortality without adequate treatment. We present the case of a 40-year-old male with a history of epilepsy treated with valproic acid and lamotrigine, previously diagnosed with dengue. Evaluation showed erythematous blisters on skin and mucosa with bleeding and desquamation, covering 10% of the body surface. The patient progressed favorably with the medical care received. Stevens-Johnson syndrome should be studied in association with arboviral diseases.
Assuntos
Dengue , Síndrome de Stevens-Johnson , Masculino , Humanos , Adulto , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/diagnóstico , Peru , Anticonvulsivantes , Ácido Valproico/uso terapêutico , Dengue/complicações , Dengue/diagnósticoRESUMO
Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are potentially life-threatening severe cutaneous adverse drug reactions. These diseases are rare, and their onset is difficult to predict because of their idiosyncratic reactivity. The Japan Severe Adverse Reactions Research Group, led by the National Institute of Health Sciences, has operated a nationwide to collect clinical information and genomic samples from patients with SJS/TEN since 2006. This study evaluated the associations of clinical symptoms with sequelae and specific causative drugs/drug groups in Japanese patients with SJS/TEN to identify clinical clues for SJS/TEN treatment and prognosis. Acetaminophen, antibiotics, and carbocisteine were linked to high frequencies of severe ocular symptoms and ocular sequelae (p < 0.05). For erythema and erosion areas, antipyretic analgesics had higher rates of skin symptom affecting <10% of the skin than the other drugs, suggesting narrower lesions (p < 0.004). Hepatic dysfunction, was common in both SJS and TEN, and antiepileptic drugs carried higher risks of hepatic dysfunction than the other drug groups (p = 0.0032). This study revealed that the clinical manifestations of SJS/TEN vary according to the causative drugs.
Assuntos
Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/etiologia , Síndrome de Stevens-Johnson/complicações , Japão/epidemiologia , Pele/patologia , Acetaminofen/efeitos adversos , OlhoRESUMO
Drug reactions affecting the vulva are understudied and underreported, with some having the potential to cause serious morbidity through long-term sequelae. We conducted a literature review to investigate the current evidence about vulval drug eruptions. We aimed to establish the extent of drug reactions affecting the vulva, identify the common culprit drugs, and review current evidence and guidelines regarding their management. The vulval involvement seen in Steven-Johnson syndrome, toxic epidermal necrolysis and fixed drug eruption forms the focus of this review, but we also summarize the current evidence regarding less common reactions.
Assuntos
Toxidermias , Síndrome de Stevens-Johnson , Feminino , Humanos , Toxidermias/etiologia , Síndrome de Stevens-Johnson/complicações , Progressão da DoençaAssuntos
Neoplasias da Mama , Lúpus Eritematoso Cutâneo , Lúpus Eritematoso Sistêmico , Síndrome de Stevens-Johnson , Humanos , Feminino , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/diagnóstico , Neoplasias da Mama/complicações , Lúpus Eritematoso Cutâneo/complicações , Lúpus Eritematoso Cutâneo/diagnóstico , Diagnóstico Diferencial , Lúpus Eritematoso Sistêmico/complicaçõesRESUMO
Reactive infectious mucocutaneous eruption (RIME) is an eruptive mucositis with varying degrees of cutaneous involvement presumed to be due to an immunologic response to various infectious pathogens. Most reported cases occur after a prodromal upper respiratory illness. We present a patient with a particularly severe case mimicking drug-induced epidermal necrolysis found to be triggered by asymptomatic norovirus infection, a virus not previously reported in association with RIME.
Assuntos
Mucosite , Norovirus , Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/etiologia , Síndrome de Stevens-Johnson/complicações , Mucosite/complicaçõesRESUMO
Toxic epidermal necrolysis and mucopolysaccharidosis are both rare diseases that pose significant airway maintenance challenges to anesthesiologists. In this report, we describe the anesthesia management in a 4-year-old male with mucopolysaccharidosis type II who developed toxic epidermal necrolysis and presented for ophthalmic surgical procedures. Combined use of propofol and ketamine with the support of high-flow nasal oxygen enabled adequate analgesia and sedation while maintaining spontaneous ventilation and airway patency. The strategy presented in this report may contribute to enhancing the safety of sedation in spontaneously breathing children with abnormal airways.
Assuntos
Anestesia , Ketamina , Mucopolissacaridose II , Propofol , Síndrome de Stevens-Johnson , Masculino , Humanos , Criança , Pré-Escolar , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/terapia , Anestesia/métodos , Mucopolissacaridose II/complicaçõesRESUMO
Stevens-Johnson syndrome and toxic epidermal necrolysis (Lyell syndrome) are rare diseases characterized by rapid blistering followed by extensive skin and mucosal exfoliation and constitutional symptoms. In most cases, drugs are the main triggers, but the etiopathogenesis of the diseases is not fully understood. Lyell syndrome is associated with a high mortality rate, reported to be around 35%. Therefore, early diagnosis requiring close interdisciplinary cooperation is essential. The diagnosis based on the clinical picture and a detailed pharmacological history should be confirmed by histopathological examination of the skin specimen, including analysis by direct immunofluorescence.
Assuntos
Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/etiologia , Síndrome de Stevens-Johnson/complicações , PatologistasRESUMO
Stevens-Johnson syndrome (SJS), also known as the multifactorial erythematous drug eruption, is a class of adverse reactions of the skin and mucous membranes primarily caused by drug allergy often involving the oral cavity, eyes, and external genital mucosa, generally accompanied by fever, and can be life-threatening in severe cases. In February 2022, the Department of Stomatology, the First Affiliated Hospital of Zhengzhou University admitted a patient with huge inflammatory hyperplasia of bilateral lingual margins secondary to SJS. Upon admission, no other obvious symptoms were observed except for tongue hyperplasia. The patient suffered from a severe adverse drug reaction caused by acetaminophen 2 months ago and was complicated by liver dysfunction and pulmonary infection. After 1 month of treatment and rehabilitation, he developed a secondary tongue mass and was subsequently admitted to Dept. of Oral and Maxillofacial Surgery Ward 2, the First Affiliated Hospital of Zhengzhou University. After completing the examination, the tongue mass was surgically removed. After a follow-up of 11 months, the patient's condition was satisfactory and no temporary discomfort was observed. The case of tongue mass secondary to SJS is extremely rare. If a stomatologist encounters a similar case, we should carefully inquire about the drug allergy history and recent medication history, and be alert to whether or not they had adverse drug reactions recently.
Assuntos
Hipersensibilidade a Drogas , Síndrome de Stevens-Johnson , Masculino , Humanos , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/tratamento farmacológico , Hiperplasia/complicações , Hiperplasia/patologia , Pele , Hipersensibilidade a Drogas/complicações , Hipersensibilidade a Drogas/patologia , LínguaRESUMO
BACKGROUND/PURPOSE: To evaluate long-term visual outcomes of Boston type I keratoprosthesis (KPro) surgery and identify risk factors for visual failure. METHODS: Single surgeon retrospective cohort study including 85 eyes of 74 patients who underwent KPro implantation to treat severe ocular surface disease, including limbal stem cell deficiency, postinfectious keratitis, aniridia and chemical burns. Procedures were performed at the Centre hospitalier de l'Université de Montréal from October 2008 to May 2012. All patients with at least 5 years of follow-up were included in the analysis, including eyes with repeated KPro. Main outcome measures were visual acuity (VA), visual failure, defined as a sustained VA worse than the preoperative VA, postoperative complications, and device retention. RESULTS: Mean follow-up was 7.2±1.3 years (±SD). Mean VA was 2.1±0.7 (logarithm of minimal angle resolution) preoperatively and 1.9±1.2 at last follow-up. In total, 2.4% of patients had VA better than 20/200 preoperatively vs. 36.5% at last follow-up. Maintenance of improved postoperative VA was seen in 61.8% of eyes at 7 years. Preoperative factors associated with visual failure were known history of glaucoma (HR=2.7 [1.2 to 5.9], P=0.02) and Stevens-Johnson syndrome (HR=7.3 [2.5 to 21.4], P<0.01). Cumulative 8-year complication rates were 38.8% retroprosthetic membrane formation, 25.9% hypotony, 23.5% new onset glaucoma, 17.6% retinal detachment, 8.2% device extrusion and 5.9% endophthalmitis. The majority (91.8%) of eyes retained the device 8 years after implantation. CONCLUSION: Nearly two-thirds of patients exhibited improved VA 7 years after KPro implantation. Preoperative risk factors for visual failure were known glaucoma and Stevens-Johnson syndrome.
Assuntos
Órgãos Artificiais , Doenças da Córnea , Glaucoma , Síndrome de Stevens-Johnson , Humanos , Córnea/cirurgia , Próteses e Implantes/efeitos adversos , Doenças da Córnea/epidemiologia , Doenças da Córnea/cirurgia , Doenças da Córnea/complicações , Estudos Retrospectivos , Síndrome de Stevens-Johnson/complicações , Órgãos Artificiais/efeitos adversos , Canadá , Glaucoma/epidemiologia , Glaucoma/etiologia , Glaucoma/cirurgia , Implantação de Prótese/efeitos adversos , Implantação de Prótese/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , SeguimentosRESUMO
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug-induced hypersensitivity reactions characterized by widespread epidermal necrosis, mucous membrane erosions, and systemic findings. We have provided our 11-year experience from a Milan, Italy tertiary hospital managing SJS/TEN, evaluating the clinical and histopathologic features plus the impact on mortality. We retrospectively analyzed 28 patients diagnosed with SJS/TEN based on the clinical and histopathologic findings, according to the classification criteria of multiple studies. We assessed the dermatographics, comorbidities, drug history, lesion characteristics, clinical findings, treatments, blood tests, and outcomes. Severity scores (SCORTEN, Re-SCORTEN, ABCD-10) were used for treatment evaluation and mortality prediction. Data were statistically analyzed, and significant factors associated with mortality were identified. We found that among the 28 patients, 89.2% had comorbidities, mainly cardiovascular diseases, and 21.4% had autoimmune disorders. All patients had received systemic therapy (46.6% monotherapy, 53.6% combination therapy), with systemic steroids (71.4%) and intravenous immunoglobulins (67.8%) being common treatments. There were complications, including systemic infections (67.9%) and septic shock (10.7%). The overall mortality rate was 17.8%. The statistical analysis indicated that malignancy, a high ABCD-10 score, and a high neutrophil-to-lymphocyte ratio were significantly associated with mortality. The extent of affected body surface area did not correlate significantly with mortality. This study provides insights into SJS/TEN management, revealing factors influencing mortality in a high-complexity tertiary hospital setting.
Assuntos
Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/epidemiologia , Síndrome de Stevens-Johnson/terapia , Síndrome de Stevens-Johnson/complicações , Estudos Retrospectivos , Centros de Atenção Terciária , Imunoglobulinas Intravenosas/uso terapêutico , ComorbidadeRESUMO
PURPOSE: To compare the clinical features and visual outcomes in children and adults with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). DESIGN: Retrospective comparative case series. METHODS: This retrospective study included 280 eyes of 140 patients (35 children and 105 adults) with SJS/TEN treated between 2010 and 2020. The primary outcome measures were the final best-corrected visual acuity (BCVA) and severity of dry eye. The secondary outcome measure was the medical and surgical therapies used. RESULTS: Among 64 eyes of children recruited in the study, acute ocular involvement was found in 58 eyes (90.6%). The chronic score in pediatric patients was significantly higher than that in adult patients (P = .004). The use of antibiotics/nonsteroidal anti-inflammatory drugs (NSAIDs) and Mycoplasma infection were the more common etiologies in children. In all, 75% of eyes in children maintained a visual acuity of 20/40 or better at a mean follow-up time of 4.3 years. The severity of dryness was comparable between the child and adult groups. The proportion of eyes undergoing amniotic membrane and oral mucosa transplantation was significantly higher in children than in adults in the chronic stage, reflecting that children exhibit much more severe complications. CONCLUSIONS: Although pediatric SJS/TEN patients have more severe ocular complications than adults, most children maintain long-term good vision. Early intervention and aggressive treatment help to preserve vision.
Assuntos
Síndromes do Olho Seco , Síndrome de Stevens-Johnson , Criança , Humanos , Adulto , Estudos Retrospectivos , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/tratamento farmacológico , Seguimentos , Síndromes do Olho Seco/diagnóstico , Síndromes do Olho Seco/tratamento farmacológico , Síndromes do Olho Seco/etiologia , Antibacterianos/uso terapêuticoRESUMO
INTRODUCTION: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome are rare severe hypersensitivity reactions that lead to epithelial sloughing. Studies investigating the chronic multisystem effects of these syndromes and assessing patients in terms of quality of life (QOL), depression, and anxiety in the pediatric population are limited. In this study, we aimed to investigate the long-term effects of these diseases from a multisystem perspective. METHOD: Sixteen pediatric patients diagnosed with SJS, TEN, and SJS/TEN overlap syndrome were evaluated between September 2020 and March 2021. Physical and eye examinations were performed. To evaluate QOL and psychological status, Children's Dermatology Life Quality Index (CDLQI), Screen for Child Anxiety-Related Emotional Disorders (SCARED), and Children's Depression Inventory (CDI) were conducted. The patients' general characteristics, symptoms, and examination findings at their first admission were retrospectively obtained from the hospital's electronic records. RESULTS: Nineteen percent of the patients were female (n = 3). There were 7 patients (44%) with the diagnosis of SJS, 5 patients (31%) with TEN, and 4 patients (25%) with SJS/TEN overlap. The median follow-up time of the subjects was 6.5 years. The most common sequelae in the chronic period were skin changes (n = 13, 81%). Hyperpigmentation was the most common skin change (n = 9, 56%). In the last evaluation, 9 cases had eye involvement. In two cases, eye examination was normal in the acute phase, while ocular involvement was present in the chronic period. In 4 (50%) patients, there was height and/or weight percentile loss. Three patients' SCARED scores and 2 patients' CDI scores were high. According to the CDLQI survey, SJS, TEN, or SJS/TEN overlap syndrome had a small to moderate effect on the QOL in the 43% (n = 6) of the patients. The ANA values of 3 patients (60%) were positive at the follow-up and negative at the first admission. CONCLUSION: SJS, TEN, and SJS/TEN overlap syndrome may cause sequelae even after a long time of the onset of the disease. Patients' QOL and psychological status can be affected negatively. Ocular symptoms may develop in the follow-up, even without involvement in the acute period. Patients with SJS, TEN, and SJS/TEN overlap syndrome should be followed up in the chronic period and approached multidisciplinary.
Assuntos
Hipersensibilidade , Síndrome de Stevens-Johnson , Humanos , Criança , Feminino , Masculino , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/diagnóstico , Qualidade de Vida , Estudos Retrospectivos , Pele , Hipersensibilidade/complicaçõesRESUMO
PURPOSE: To clarify the importance of administering topical steroids for the treatment of Stevens-Johnson syndrome (SJS) / toxic epidermal necrolysis (TEN) with ocular involvement in the acute phase. DESIGN: Retrospective case series. METHODS: Using the medical records of acute SJS/TEN patients treated at the Kyoto Prefectural University of Medicine Hospital, Kyoto, Japan, between July 2006 and July 2017, the ocular findings, topical steroid dosage, systemic steroid dosage, and ocular sequelae were retrospectively examined. The level of cytokines in tear fluid and serum samples was also analyzed. RESULTS: This study involved 13 cases. In 10 cases in whom the clinical courses were recorded before the start of steroid therapy, the mean acute ocular severity score (AOSS: 3 = very severe; 2 = severe; 1 = mild; 0 = none) was 2.8 ± 0.4 points in the severest phase. The mean systemic steroid dose after steroid pulse therapy was 694 ± 386 mg and the mean topical steroid (0.1% betamethasone eye drop and ointment) dose was 13.4 ± 3.3 times daily in the severest phase. Analysis of cytokine levels of 4 cases showed that a cytokine storm occurred in the tear fluid after the steroid pulse therapy. At final follow-up, 16 eyes of 8 patients had a logMAR visual acuity of ≤0, and no serious ocular sequelae were observed. CONCLUSIONS: In patients with SJS/TEN, ocular surface inflammation remains strong even after systemic inflammation has improved post steroid pulse therapy, thus suggesting that both systemic and topical steroid therapy should be administered appropriately.
Assuntos
Betametasona , Glucocorticoides , Síndrome de Stevens-Johnson , Betametasona/administração & dosagem , Betametasona/uso terapêutico , Humanos , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/tratamento farmacológico , Administração Tópica , Estudos Retrospectivos , Anti-Inflamatórios , Acuidade Visual , Glucocorticoides/administração & dosagem , Pulsoterapia , Oftalmopatias/etiologia , Masculino , Feminino , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , IdosoRESUMO
PURPOSE: This case describes the successful visual restoration of a patient with end-stage Stevens-Johnson syndrome (SJS) with a severely keratinized ocular surface. METHODS: This study is a case report. RESULTS: A 67-year-old man with SJS secondary to allopurinol sought visual rehabilitation options. His ocular surface was severely compromised from sequelae of chronic SJS, leaving him with light perception vision bilaterally. The left eye was completely keratinized with severe ankyloblepharon. The right eye had failed penetrating keratoplasty, limbal stem cell deficiency, and a keratinized ocular surface. The patient declined both a Boston type 2 keratoprosthesis and a modified osteo-odonto keratoprosthesis. Therefore, a staged approach was pursued with (1) systemic methotrexate to control ocular surface inflammation, (2) minor salivary gland transplant to increase ocular surface lubrication, (3) lid margin mucous membrane graft to reduce keratinization, and finally, (4) Boston type 1 keratoprosthesis for visual restoration. After minor salivary gland transplant and mucous membrane graft, the Schirmer score improved from 0 mm to 3 mm with improvement in ocular surface keratinization. This approach successfully restored the vision to 20/60, and the patient has retained the keratoprosthesis for over 2 years. CONCLUSIONS: Sight restoration options are limited in patients with end-stage SJS with a keratinized ocular surface, aqueous and mucin deficiency, corneal opacification, and limbal stem cell deficiency. This case demonstrates successful ocular surface rehabilitation and vision restoration in such a patient through a multifaceted approach that resulted in successful implantation and retention of a Boston type 1 keratoprosthesis.