RESUMEN
OBJECTIVES: To evaluate the long-term benefits of tear-exchangeable, limbal-rigid contact lens (CL) wear therapy in patients with Stevens-Johnson syndrome (SJS)-associated ocular sequelae. METHODS: This retrospective study evaluated 50 eyes of 41 SJS patients (15 men and 26 women) who underwent limbal-rigid CL wear therapy for more than 2 years post fitting. Ocular sequelae (i.e., conjunctival hyperemia, corneal neovascularization, and upper tarsus scarring) before fitting and at 3 months, 6 months, 12 months, and annually after initiating CL wear therapy were evaluated and then graded on a severity score (range: 0-3, maximum score: 3). Moreover, visual acuity (VA) at immediately post initiating CL wear therapy was evaluated. RESULTS: The mean follow-up period was 4.3±1.1 years. Compared with before fitting, the mean conjunctival hyperemia score improved from 1.14 to 0.86 at 3 months of CL wear therapy ( P <0.01) and was maintained thereafter; the mean corneal neovascularization score improved from 2.10 to 1.98 at 3 months of CL wear therapy, with no deterioration of the score observed in all cases at the final follow-up examination, and mean VA (log of minimum angle of resolution) improved from 1.60 to 1.04 at immediately post initiating CL wear therapy ( P <0.01). CONCLUSIONS: Limbal-rigid CL wear therapy can provide long-term ocular surface stabilization and improved VA in SJS patients.
Asunto(s)
Conjuntivitis , Lentes de Contacto , Enfermedades de la Córnea , Neovascularización de la Córnea , Hiperemia , Síndrome de Stevens-Johnson , Masculino , Humanos , Femenino , Enfermedades de la Córnea/terapia , Enfermedades de la Córnea/complicaciones , Síndrome de Stevens-Johnson/terapia , Síndrome de Stevens-Johnson/complicaciones , Neovascularización de la Córnea/terapia , Neovascularización de la Córnea/complicaciones , Estudios Retrospectivos , Progresión de la EnfermedadRESUMEN
Toxic epidermal necrolysis (TEN) is a potentially life-threatening muco-cutaneous disease, largely caused by an idiosyncratic reaction to medication or infectious disease, and is characterised by acute necrosis of the epidermis. No definitive consensus regarding the treatment of TEN has been agreed. A 60-year-old woman, diagnosed with multiple myeloma three months prior, was admitted with signs of TEN to the intensive care burns unit. She had been given ciprofloxacin to treat a urinary tract infection. She complained of malaise and pain, with maculopapular and bullous eruptions over her whole body on the third day of ciprofloxacin administration. Her supportive cares included intravenous immunoglobulins, pain control with analgesics, wound care, nutrition, and fluid support. Hyperbaric oxygen treatment (HBOT) was added on the second day of admission. The patient underwent 5 sessions of HBOT at 243.1 kPa (2.4 atmospheres absolute). Desquamation was noted to stop after the first session of HBOT and re-epithelisation commenced rapidly. The patient was discharged from the burn unit after 14 days of hospital admission. Improvement in this case was temporally related to the initiation of HBOT.
Asunto(s)
Oxigenoterapia Hiperbárica , Síndrome de Stevens-Johnson , Cuidados Críticos , Femenino , Humanos , Persona de Mediana Edad , Oxígeno , Síndrome de Stevens-Johnson/terapiaRESUMEN
Significance: Toxic epidermal necrolysis (TEN) and Steven-Johnson syndrome (SJS) are potentially fatal acute mucocutaneous vesiculobullous disorders. Evidence to date suggests that outcomes for patients with both TEN and SJS are largely dependent on stopping the causative agent, followed by supportive care and appropriate wound management in a specialized burns unit. These are life-threatening conditions characterized by widespread full-thickness cutaneous and mucosal necrosis. This article outlines the approach to holistic management of such patients, in a specialized unit, highlighting various practical aspects of wound care to prevent complications such as infection, mucosal and adhesions, and ocular scaring. Recent Advances: There is improved understanding of pain and morbidity with regard to the type and frequency of dressing changes. More modern dressings, such as nanocrystalline, are currently favored as they may be kept in situ for longer periods. The most recent evidence on systemic agents, such as corticosteroids and cyclosporine, and novel treatments, are also discussed. Critical Issues: Following cessation of the culprit trigger, management in a specialized burns unit is the most important management step. It is now understood that a multidisciplinary team is essential in the care of these patients. Following admission of such patients, dermatology, ear, nose, and throat surgery, ophthalmology, urology, colorectal surgery, and gynecology should all be consulted to prevent disease sequelae. Future Directions: Looking forward, research is aimed at achieving prospective data on the efficacy of systemic immunomodulating agents and dressing types. Tertiary centers with burns units should develop policies for such patients to ensure that the relevant teams are consulted promptly to avoid mucocutaneous complications.
Asunto(s)
Salud Holística , Apoyo Nutricional/métodos , Cuidados Paliativos/métodos , Trasplante de Piel/métodos , Síndrome de Stevens-Johnson/terapia , Corticoesteroides/farmacología , Corticoesteroides/uso terapéutico , Animales , Vendajes , Unidades de Quemados/organización & administración , Ciclosporina/farmacología , Ciclosporina/uso terapéutico , Hospitalización , Humanos , Inmunoglobulinas Intravenosas/farmacología , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/farmacología , Factores Inmunológicos/uso terapéutico , Tiempo de Internación , Grupo de Atención al Paciente/organización & administración , Piel/efectos de los fármacos , Piel/inmunología , Síndrome de Stevens-Johnson/epidemiología , Síndrome de Stevens-Johnson/etiología , Porcinos , Centros de Atención Terciaria/organización & administración , Trasplante Heterólogo/métodos , Resultado del Tratamiento , Cicatrización de Heridas/efectos de los fármacos , Cicatrización de Heridas/inmunologíaRESUMEN
RESUMEN La necrolisis epidérmica tóxica es una reacción cutánea adversa de tipo inmunológico secundaria en la mayor parte de los casos a la administración de un fármaco. La necrolisis epidérmica tóxica, el síndrome de Steven Johnson y el eritema exudativo multiforme forman parte del mismo espectro de enfermedad. La mortalidad de la necrolisis epidérmica tóxica es alrededor del 30%. La fisiopatología de la necrolisis epidérmica tóxica es semejante en muchos aspectos a la de las quemaduras dérmicas superficiales. La afectación mucosa del epitelio ocular y genital se asocia con secuelas graves si no se trata de forma temprana. Se acepta en general que los pacientes con necrolisis epidérmica tóxica son tratados mejor en unidades de grandes quemados, donde existe experiencia en el manejo de enfermos con pérdida cutánea extensa. El tratamiento es de soporte, eliminación y cobertura con derivados biosintéticos de la piel de las zonas afectadas, tratamiento de la afectación mucosa, y tratamiento inmunosupresor específico. De los tratamientos ensayados sólo se usa actualmente en la mayor parte de los centros la inmunoglobulina G y la ciclosporina A, aun cuando no existe evidencia sólida para recomendar ningún tratamiento específico. Entre los aspectos particulares del tratamiento de esta enfermedad se encuentra la prevención de secuelas relacionadas con la formación de sinequias, los cuidados oculares para prevenir secuelas graves que pueden conducir a la ceguera, y el tratamiento específico inmunosupresor. Un mejor conocimiento de los principios del manejo de la necrolisis epidérmica tóxica llevará a un mejor manejo de la enfermedad, a una mayor supervivencia y una menor prevalencia de las secuelas.
ABSTRACT Toxic epidermal necrolysis is an adverse immunological skin reaction secondary in most cases to the administration of a drug. Toxic epidermal necrolysis, Stevens-Johnson syndrome, and multiform exudative erythema are part of the same disease spectrum. The mortality rate from toxic epidermal necrolysis is approximately 30%. The pathophysiology of toxic epidermal necrolysis is similar in many respects to that of superficial skin burns. Mucosal involvement of the ocular and genital epithelium is associated with serious sequelae if the condition is not treated early. It is generally accepted that patients with toxic epidermal necrolysis are better treated in burn units, which are experienced in the management of patients with extensive skin loss. Treatment includes support, elimination, and coverage with biosynthetic derivatives of the skin in affected areas, treatment of mucosal involvement, and specific immunosuppressive treatment. Of the treatments tested, only immunoglobulin G and cyclosporin A are currently used in most centers, even though there is no solid evidence to recommend any specific treatment. The particular aspects of the treatment of this disease include the prevention of sequelae related to the formation of synechiae, eye care to prevent serious sequelae that can lead to blindness, and specific immunosuppressive treatment. Better knowledge of the management principles of toxic epidermal necrolysis will lead to better disease management, higher survival rates, and lower prevalence of sequelae.
Asunto(s)
Humanos , Síndrome de Stevens-Johnson/fisiopatología , Enfermedad Crítica , Inmunosupresores/uso terapéutico , Inmunoglobulina G/uso terapéutico , Tasa de Supervivencia , Síndrome de Stevens-Johnson/complicaciones , Síndrome de Stevens-Johnson/terapia , Ciclosporina/uso terapéutico , Progresión de la EnfermedadRESUMEN
Toxic epidermal necrolysis (TEN) is a severe adverse drug reaction associated with the separation of skin and mucous membranes at the dermal-epidermal junction. Although it is rare, many treatments have been trialed because of its high mortality rate. Active interventions performed to date include the use of systemic corticosteroids, intravenous immunoglobulins (IVIg), cyclosporine, plasmapheresis, anti-tumor necrosis factor drugs and N-acetylcysteine, but none has been established as the most effective therapy. IVIg and short-term high-dose corticosteroids were regarded as the most promising treatments for TEN in a comprehensive review of all reported TEN cases from 1975-2003. When used with an appropriate dose and timing, the beneficial effects of IVIg can be maximized. Although no randomized controlled trials have been conducted, cyclosporine and plasmapheresis are considered to be beneficial. As no gold standard for active intervention for TEN has been established, the choice of treatment relies partly on the available guidelines and the experience of the dermatologist. There is still much to be investigated regarding the pathogenesis of TEN, and new findings may contribute to the identification of an effective active intervention strategy.
Asunto(s)
Glucocorticoides/administración & dosificación , Inmunoglobulinas Intravenosas/administración & dosificación , Síndrome de Stevens-Johnson/terapia , Acetilcisteína/administración & dosificación , Ciclosporina/administración & dosificación , Etanercept/administración & dosificación , Humanos , Inmunosupresores/administración & dosificación , Infliximab/administración & dosificación , Plasmaféresis , Talidomida/administración & dosificación , Factor de Necrosis Tumoral alfa/antagonistas & inhibidoresRESUMEN
PURPOSE: Toxic epidermal necrolysis (TEN) is a rare, life-threatening, drug-induced, mucocutaneous disease, which can severely affect the ocular surface. The purpose of this study was to investigate the efficacy of plasmapheresis, human IV immunoglobulins (IVIg), and autologous serum (AS) eyedrops in the treatment of the severe acute ocular complications of TEN. METHODS: A retrospective chart review of all patients admitted to the Burn Unit, Azienda Ospedaliero-Universitaria-Sassari, Sassari, Italy, from 2009 to 2015, identified 9 patients (2 men, 7 women; mean age 63.8 ± 24.7 years) with TEN. Bilateral, acute ocular surface complications were observed in 7 (78%) patients; 3 showed catarrhal conjunctivitis, whereas 4 had severe pseudomembranous conjunctivitis and corneal ulcers. RESULTS: All patients with TEN were immediately treated with plasmapheresis and human IVIg, which produced a marked improvement in the patients' general condition. In the 3 with catarrhal conjunctivitis, preservative-free artificial tears and topical antibiotics were beneficial. In the 4 with severe pseudomembranous conjunctivitis and corneal ulcers, treatment with AS eyedrops resulted in corneal and conjunctival epithelium healing over 3-6 weeks. After a minimum follow-up of at least 12 months, there were minimal/mild residual signs and symptoms of dry eye. CONCLUSIONS: Plasmapheresis and IVIg may be life-saving and contribute to reduce ocular surface inflammation in TEN. Autologous serum eyedrops, prepared after plasmapheresis completion and IVIg infusion, may be helpful in the management of the severe acute ocular complications of TEN.
Asunto(s)
Conjuntivitis/terapia , Enfermedades de la Córnea/terapia , Enfermedades de los Párpados/terapia , Inmunoglobulinas Intravenosas/uso terapéutico , Plasmaféresis/métodos , Suero , Síndrome de Stevens-Johnson/terapia , Enfermedad Aguda , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Conjuntivitis/diagnóstico , Conjuntivitis/etiología , Enfermedades de la Córnea/diagnóstico , Enfermedades de la Córnea/etiología , Enfermedades de los Párpados/diagnóstico , Enfermedades de los Párpados/etiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Soluciones Oftálmicas , Estudios Retrospectivos , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/etiologíaRESUMEN
INTRODUCTION: The diffuse epidermal exfoliation seen in Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) is similar to skin loss in second degree burns, and many of these patients are referred for treatment at burn centers. Treatment can differ markedly from center to center, and mortality can range from 25% to 70%, including a considerable morbidity. However, our experience over a 15-year period from 2000 to 2015 with 40 patients found a mortality rate of only 10% (4/40). The purpose of this paper is to discuss our treatment algorithm as a model for other centers treating SJS/TENs patients. METHODS: Records were reviewed for all patients admitted to the LAC+USC burn unit between 2000 and 2015 and 40 patients were identified with biopsy-proven SJS or TENS. These cases were reviewed for age, gender, initial and greatest TBSA, causative drug, pre-existing medical conditions, and morbidity and mortality. All data were entered into the SPSS statistical software package and all statistical analyses were performed using this program. RESULTS: Our treatment algorithm focused on early referral to a specialty burn unit, immediate discontinuation of the offending drug, fluid resuscitation, nutritional supplementation, and meticulous wound care. Average time to transfer to a burn unit was 3.36 days. Silver-releasing antimicrobial dressings were applied to the affected skin surface and changed every 3 days. Mupirocin coated petroleum gauze was used for facial involvement. Steroids were tapered and discontinued if initiated at an outside facility (58% of patients), and starting after 2001, all patients received a course of IVIG. All patients received fluid resuscitation and the majority received supplemental tube feedings (69%). Average length of total stay was 17.1 days and length of ICU stay 15.9 days. While 44% were transferred to another facility for further rehabilitative care, 37% of patients discharge to home. In patients discharged home with complete resolution of skin lesions, time to healing was an average of 14 days. DISCUSSION: With our 10% mortality rate in 40 patients, our study represents a relatively large study population while maintaining a relatively low mortality rate. The demographic data from our study largely aligns with the existing literature, and we therefore feel that our low mortality rate is due to our treatment algorithm, rather than to a less severe pathology in our patient population. This claim is supported by a standard mortality ratio of 1.68. This ratio proves a significantly improved mortality than would be expected based on disease severity on admission.
Asunto(s)
Algoritmos , Antibacterianos/uso terapéutico , Antiinfecciosos Locales/uso terapéutico , Nutrición Enteral , Fluidoterapia , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Síndrome de Stevens-Johnson/terapia , Administración Cutánea , Alopurinol/efectos adversos , Antibacterianos/efectos adversos , Anticonvulsivantes/efectos adversos , Vendajes , Superficie Corporal , Unidades de Quemados , Protocolos Clínicos , Femenino , Supresores de la Gota/efectos adversos , Hospitalización , Humanos , Unidades de Cuidados Intensivos , Tiempo de Internación , Masculino , Persona de Mediana Edad , Mupirocina/uso terapéutico , Transferencia de Pacientes , Poliésteres/uso terapéutico , Polietilenos/uso terapéutico , Centros de Rehabilitación , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Síndrome de Stevens-Johnson/etiología , Síndrome de Stevens-Johnson/mortalidadRESUMEN
Toxic epidermal necrolysis is an adverse immunological skin reaction secondary in most cases to the administration of a drug. Toxic epidermal necrolysis, Stevens-Johnson syndrome, and multiform exudative erythema are part of the same disease spectrum. The mortality rate from toxic epidermal necrolysis is approximately 30%. The pathophysiology of toxic epidermal necrolysis is similar in many respects to that of superficial skin burns. Mucosal involvement of the ocular and genital epithelium is associated with serious sequelae if the condition is not treated early. It is generally accepted that patients with toxic epidermal necrolysis are better treated in burn units, which are experienced in the management of patients with extensive skin loss. Treatment includes support, elimination, and coverage with biosynthetic derivatives of the skin in affected areas, treatment of mucosal involvement, and specific immunosuppressive treatment. Of the treatments tested, only immunoglobulin G and cyclosporin A are currently used in most centers, even though there is no solid evidence to recommend any specific treatment. The particular aspects of the treatment of this disease include the prevention of sequelae related to the formation of synechiae, eye care to prevent serious sequelae that can lead to blindness, and specific immunosuppressive treatment. Better knowledge of the management principles of toxic epidermal necrolysis will lead to better disease management, higher survival rates, and lower prevalence of sequelae.
La necrolisis epidérmica tóxica es una reacción cutánea adversa de tipo inmunológico secundaria en la mayor parte de los casos a la administración de un fármaco. La necrolisis epidérmica tóxica, el síndrome de Steven Johnson y el eritema exudativo multiforme forman parte del mismo espectro de enfermedad. La mortalidad de la necrolisis epidérmica tóxica es alrededor del 30%. La fisiopatología de la necrolisis epidérmica tóxica es semejante en muchos aspectos a la de las quemaduras dérmicas superficiales. La afectación mucosa del epitelio ocular y genital se asocia con secuelas graves si no se trata de forma temprana. Se acepta en general que los pacientes con necrolisis epidérmica tóxica son tratados mejor en unidades de grandes quemados, donde existe experiencia en el manejo de enfermos con pérdida cutánea extensa. El tratamiento es de soporte, eliminación y cobertura con derivados biosintéticos de la piel de las zonas afectadas, tratamiento de la afectación mucosa, y tratamiento inmunosupresor específico. De los tratamientos ensayados sólo se usa actualmente en la mayor parte de los centros la inmunoglobulina G y la ciclosporina A, aun cuando no existe evidencia sólida para recomendar ningún tratamiento específico. Entre los aspectos particulares del tratamiento de esta enfermedad se encuentra la prevención de secuelas relacionadas con la formación de sinequias, los cuidados oculares para prevenir secuelas graves que pueden conducir a la ceguera, y el tratamiento específico inmunosupresor. Un mejor conocimiento de los principios del manejo de la necrolisis epidérmica tóxica llevará a un mejor manejo de la enfermedad, a una mayor supervivencia y una menor prevalencia de las secuelas.
Asunto(s)
Enfermedad Crítica , Inmunosupresores/uso terapéutico , Síndrome de Stevens-Johnson/fisiopatología , Ciclosporina/uso terapéutico , Progresión de la Enfermedad , Humanos , Inmunoglobulina G/uso terapéutico , Síndrome de Stevens-Johnson/complicaciones , Síndrome de Stevens-Johnson/terapia , Tasa de SupervivenciaRESUMEN
PURPOSE: To describe a new grading system and associated treatment guidelines for the acute ocular manifestations of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). DESIGN: Prospective case series. PARTICIPANTS: Seventy-nine consecutive patients (158 eyes) evaluated and treated for acute ocular involvement in SJS or TEN during hospitalization. METHODS: Photographic and chart review of acute ocular findings, interventions received, and outcomes with regard to visual acuity, dry eye symptoms, and scarring sequelae at least 3 months after the acute illness. MAIN OUTCOME MEASURES: Visual acuity, dry eye severity, and scarring of the ocular surface and eyelids were assessed after follow-up of at least 3 months. RESULTS: Cases graded as mild or moderate were managed medically. All had best-corrected visual acuity (BCVA) of 20/20, no dry eye symptoms, and no scarring sequelae. Cases graded as severe or extremely severe were treated with urgent amniotic membrane transplantation (AMT) in addition to medical management. Severe cases all had BCVA of 20/20 and mild or no dry eye problems. Five of 28 patients had mild tarsal conjunctival scarring. No other scarring sequelae occurred. Nine of the 10 extremely severe cases had BCVA of 20/20 (1 was 20/30). Three of 10 had moderate scarring of the tarsal conjunctiva and lid margins and also moderate dry eyes with severe photophobia. Seven of 10 had only mild or no dry eye symptoms and scarring sequelae. CONCLUSIONS: This grading system facilitates decision making in the evaluation and management of the acute ocular manifestations of SJS and TEN. Mild and moderate cases have a low risk of significant scarring or visual sequelae and may be monitored and treated medically if not worsening. Severe and extremely severe cases should receive urgent AMT to decrease the risk of scarring and visual sequelae.
Asunto(s)
Amnios/trasplante , Antibacterianos/uso terapéutico , Glucocorticoides/uso terapéutico , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/terapia , Enfermedad Aguda , Terapia Combinada , Ciclosporina/uso terapéutico , Dexametasona/uso terapéutico , Síndromes de Ojo Seco/diagnóstico , Enfermedades de los Párpados/diagnóstico , Femenino , Fluorofotometría , Fluoroquinolonas/uso terapéutico , Guías como Asunto , Humanos , Persona de Mediana Edad , Moxifloxacino , Estudios Prospectivos , Índice de Severidad de la Enfermedad , Síndrome de Stevens-Johnson/clasificación , Tobramicina/uso terapéutico , Agudeza Visual/fisiologíaRESUMEN
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a systemic disease that can be associated with debilitating acute and chronic complications across multiple organ systems. As patients with acute SJS/TEN are often treated in a burn intensive care unit (BICU), we surveyed burn centers across the United States to determine their approach to the care of these patients. The goal of our study was to identify best practices and possible variations in the care of patients with acute SJS/TEN. We demonstrate that the method of diagnosis, use of systemic therapies, and involvement of subspecialists varied significantly between burn centers. Beyond supportive care provided to every patient, our data highlights a lack of standardization in the acute care of patients with SJS/TEN. A comprehensive guideline for the care of patients with acute SJS/TEN is indicated.
Asunto(s)
Unidades de Quemados , Prestación Integrada de Atención de Salud/normas , Unidades de Cuidados Intensivos , Síndrome de Stevens-Johnson/terapia , Enfermedad Aguda , Adulto , Niño , Femenino , Disparidades en Atención de Salud , Humanos , Masculino , Educación del Paciente como Asunto/normas , Síndrome de Stevens-Johnson/diagnóstico , Estados UnidosRESUMEN
PURPOSE: To evaluate the results of Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) scleral lens treatment on visual acuity and function in patients with ocular symptoms of chronic Stevens-Johnson syndrome (SJS). DESIGN: Retrospective interventional case series. METHODS: setting: Single multi-specialty institutional practice. study population: A chart review from July 2009 to July 2013 identified 19 patients with ocular symptoms from chronic SJS who were referred for PROSE fitting evaluation. Three patients deemed appropriate candidates were excluded because they were lost to follow-up during the fitting process. Only 1 eye was fitted in 4 patients because anatomic changes prohibited PROSE fitting in the fellow eye. Another patient chose to have PROSE fitting only in 1 eye. A total of 27 eyes of 16 patients who completed PROSE fitting were included in this study. intervention: PROSE scleral lens fitting. outcome measures: Visual acuity and visual function were assessed before and after PROSE fitting using Snellen acuity and Ocular Surface Disease Index (OSDI) survey. The OSDI survey is a validated questionnaire that assesses ocular surface disease in the context of vision-related function, ocular symptoms, and environmental triggers. RESULTS: Visual acuity improved from 0.43 ± 0.35 logMAR pre-PROSE to 0.14 ± 0.22 logMAR post-PROSE (P = .0007) in SJS patients. OSDI scores improved from 70.4 ± 19.0 pre-PROSE to 37.4 ± 23.2 post-PROSE (P = .0002) in the same cohort. CONCLUSION: The results of this study show that PROSE treatment is a viable option for improving visual acuity and function in SJS patients who failed conventional treatment.
Asunto(s)
Conjuntivitis/terapia , Lentes de Contacto , Esclerótica , Síndrome de Stevens-Johnson/terapia , Adulto , Enfermedad Crónica , Conjuntivitis/fisiopatología , Ecosistema , Femenino , Humanos , Masculino , Ajuste de Prótesis , Estudios Retrospectivos , Síndrome de Stevens-Johnson/fisiopatología , Encuestas y Cuestionarios , Resultado del Tratamiento , Agudeza Visual/fisiologíaRESUMEN
BACKGROUND: Drugs are by far the most common cause of toxic epidermal necrolysis (TEN), but unidentified drugs and chemicals present in herbal and traditional medications may also be responsible for this reaction, which manifests as widespread epidermal detachment of the skin and mucous membrane. In contexts in which a subject has used an herbal or traditional medication, it is very difficult to diagnose the condition, identify the offending agent, and prevent the disease from recurring. METHODS: This prospective study was conducted at a tertiary burn center between July 2004 and October 2012. All patients were referred to this unit by the local department of medicine at least one week after the eruption of vesicles. On arrival, all patients demonstrated a severe form of disease with features of sepsis and electrolyte imbalance (SCORTEN scores of ≥4). All non-fatal cases were followed to observe long-term sequelae and recurrences. RESULTS: About 34% of patients had developed the condition after ingesting traditional herbal medications and were unable to identify the responsible drug by name. Nineteen (66%) of the 29 patients referred to the unit with TEN died within the first week after being transferred. Nine patients achieved complete recovery, and one developed corneal haziness and alopecia. One patient experienced recurrence within three months but recovered. CONCLUSIONS: Illiteracy and financial vulnerability were major factors in driving patients towards the use of traditional medications, which were often prepared and preserved improperly. Mortality and other complications could be reduced by the prompt recognition of the condition, immediate withdrawal of the culprit drug, and quick referral to proper care.
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Países en Desarrollo , Materia Medica/efectos adversos , Preparaciones de Plantas/efectos adversos , Síndrome de Stevens-Johnson/etiología , Adolescente , Adulto , Bangladesh , Niño , Escolaridad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pobreza , Estudios Prospectivos , Recurrencia , Síndrome de Stevens-Johnson/terapia , Poblaciones Vulnerables , Adulto JovenRESUMEN
IMPORTANCE: Fractionated, ultrapulsed carbon dioxide (CO2) laser therapy is a powerful tool for the treatment of scars. Common adverse effects of this therapeutic modality have been previously documented. We describe 2 unreported adverse effects of ultrapulsed CO2 laser treatment of mature scars in a patient previously treated with silver-impregnated dressings. OBSERVATIONS: A teenage survivor of toxic epidermal necrolysis presented with faint but diffuse dyschromia clinically and histologically consistent with localized argyria secondary to silver-impregnated dressings used years earlier. The patient was subsequently treated with fractionated CO2 for her scarring, but her hyperpigmentation worsened with each treatment. A subsequent biopsy specimen revealed a zone of dystrophic calcification with adjacent pseudo-ochronotic fibers that were not appreciated on biopsy specimens taken before CO2 laser treatment, suggesting unique complications not previously reported. CONCLUSIONS AND RELEVANCE: We present 2 unique complications secondary to ultrapulsed, fractionated CO2 laser treatment in a patient previously treated with silver-impregnated dressings: (1) the appearance of pseudo-ochronotic fibers in areas of worsening pigmentation and (2) evidence of dystrophic calcification limited to columns of fractionated laser ablation. Therefore, a history of argyria or treatment with silver-impregnated dressings should be considered before treatment with fractionated CO2 lasers.
Asunto(s)
Argiria/etiología , Calcinosis/etiología , Cicatriz Hipertrófica/patología , Cicatriz Hipertrófica/radioterapia , Láseres de Gas/efectos adversos , Terapia por Luz de Baja Intensidad/efectos adversos , Adolescente , Argiria/patología , Vendajes/efectos adversos , Biopsia con Aguja , Calcinosis/patología , Calcinosis/terapia , Cicatriz Hipertrófica/etiología , Femenino , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Terapia por Luz de Baja Intensidad/métodos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Plata/efectos adversos , Síndrome de Stevens-Johnson/complicaciones , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/terapia , Resultado del TratamientoRESUMEN
PURPOSE: Toxic epidermal necrolysis (TEN) is a devastating form of Stevens-Johnson syndrome (SJS) with acute and chronic ocular complications. We present a novel aggressive combination strategy, termed "Triple-TEN", for the management of acute ocular TEN designed to minimize the risk of chronic, blinding sequelae. METHODS: Two patients with life-threatening TEN accompanied by severe ocular surface defects and fulminant symblephara formation underwent "Triple-TEN" management of their acute ocular disease under aseptic techniques in the critical care setting, after failed treatment with intensive topical therapy and surgical division of symblephara. The Triple-TEN protocol comprises (1) subconjunctival triamcinolone (Kenalog 20 mg) administered into each of the fornices to curb the local inflammatory response without compromising systemic immunity, (2) placement of amniotic membrane tissue mounted on a polycarbonate skirt (ProKera) over the corneal and limbal regions to facilitate reepithelialization of the ocular surface, and (3) insertion of a steeply curved acrylic scleral shell spacer (Technovent, SC21) to vault the lids away from the globe providing a barrier to symblephara formation. RESULTS: In both cases, ocular surface inflammation resolved within 4 weeks with no progression of conjunctival cicatrization or evidence of limbal epithelial stem cell failure at 1 year follow-up. There were no long-term complications of the Triple-TEN regimen. CONCLUSIONS: Aggressive treatment with the Triple-TEN protocol for acute ocular TEN resistant to first-line therapy, may help prevent long-term blinding sequelae.
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Apósitos Biológicos , Conjuntivitis/terapia , Glucocorticoides/uso terapéutico , Prótesis e Implantes , Síndrome de Stevens-Johnson/complicaciones , Síndrome de Stevens-Johnson/terapia , Triamcinolona Acetonida/uso terapéutico , Resinas Acrílicas , Enfermedad Aguda , Adolescente , Terapia Combinada , Conjuntivitis/etiología , Enfermedades de los Párpados/prevención & control , Femenino , Humanos , Persona de Mediana Edad , Cemento de Policarboxilato , Síndrome de Stevens-Johnson/etiologíaRESUMEN
Toxic epidermal necrolysis (TEN) is a severe mucocutaneous drug-induced syndrome that causes massive keratinocyte apoptosis and therefore hydro-electrolytic disorders and systemic infection. TEN approximately affects one to two cases per million per year. Mortality rate may reach thirty percent of cases. Thus, TEN constitutes a therapeutic emergency at diagnosis. Typically, clinical examination shows a mucocutaneous detachment involving more than thirty percent of body area. Definitive diagnosis is made on cutaneous biopsy with histological exam that shows the blister of necrotic keratinocytes. Main differential diagnosis are acute staphylococcus epidermis, acute generalized exanthematous pustulosis, linear IgA bullous dermatosis, paraneoplastic pemphigus, bullous fixed pigmented erythema, acute lupus erythematosus. In the early days, SCORTEN gives a good estimation and is now widely used as prognostic score. Drugs are generally considered as the main etiology of TEN but in some cases bacterial or viral infections could be involved. Physiopathology remains unclear even if recent advances have reported the possible implication of immune pathways based on activation of T and NK cells. Treatment of TEN requires to be instituted as soon as the diagnosis is made and the patient is preferentially referred to a specialized unit. Supportive care consist of covering areas of cutaneous detachment. No other therapy has demonstrated its efficiency, but high-dose intravenous immunoglobulin might improve the prognosis.
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Síndrome de Stevens-Johnson/terapia , Acetilcisteína/uso terapéutico , Corticoesteroides/uso terapéutico , Ciclosporina/uso terapéutico , Humanos , Plasmaféresis , Pronóstico , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/etiología , Factor de Necrosis Tumoral alfa/antagonistas & inhibidoresRESUMEN
UNLABELLED: Toxic epidermal necrolysis (TEN) is a rare condition with potentially high mortality and involves severe exfoliative disease of the skin and mucous membranes induced by drugs. The reported fatality of TEN varies widely from 20% to 60%. The technique for TEN wound coverage described in this article involves the use of various dressings. PATIENTS AND METHODS: Nine women with histologically confirmed TEN (>30% total body surface area, TBSA) were treated at our burn intensive care unit. All patients received hydrotherapy and wounds were covered with Aquacel Ag and Vaseline gauzes onlay. Following this, elastic cotton bandage was wrapped around the dressing. The dressing was changed and the wound evaluated twice a week. Efficacy was established by the wound achieving>or=95% re-epithelialisation of the study area. RESULTS: The mean age was 60.1 years (range from 7 to 88 years). The percentage of body surface area affected by epidermal slough ranged from 30% to 85% TBSA, with a mean of 51%. One patient expired due to severe sepsis on day 3. Eight patients achieved over 95% wound healing. All wounds healed well without the need for skin grafting. However, two of them expired on day 14 and day 20 because of pneumonia and retention of carbon dioxide, respectively. The average duration to achieve 95% wound healing was 10.4 days in eight cases (range from 7 to 14 days). No adverse reactions were noted. CONCLUSION: Aquacel Ag dressing can be easily removed during hydrotherapy. The wound pain is reduced. By changing the dressing just twice a week, we were able to evaluate the wound directly, decrease the odour and increase the quality of life of the patients. In addition, lower frequency of dressing changes decreases the manpower requirements and is cost effective. Use of Aquacel Ag with Vaseline gauze is a good alternative for the management of TEN wounds.
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Carboximetilcelulosa de Sodio/uso terapéutico , Apósitos Oclusivos , Vaselina/uso terapéutico , Síndrome de Stevens-Johnson/terapia , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Femenino , Humanos , Hidroterapia , Persona de Mediana Edad , Síndrome de Stevens-Johnson/patología , Resultado del Tratamiento , Cicatrización de Heridas , Adulto JovenRESUMEN
BACKGROUND: Toxic epidermal necrolysis syndrome is a devastating disease, with mortality rates ranging between 20 and 60 percent. This study evaluated an alternative treatment approach using antishear wound care and compared outcomes using the severity of illness score for toxic epidermal necrolysis syndrome (SCORTEN) system. METHODS: Records of 48 patients with a histopathologic diagnosis of toxic epidermal necrolysis syndrome treated with burn equivalent critical care and antishear wound care from September of 1985 to April of 2004 were reviewed. Observed mortality data were compared with those expected using the SCORTEN, and the standardized mortality ratio was calculated. RESULTS: The overall mortality rate was 27 percent. Factors affecting mortality were advancing age, time to burn unit admission, multisystem organ failure, and presence of comorbidities (p = 0.02, p = 0.02, p < 0.001, and p = 0.003, respectively). Chronic renal insufficiency and malignancy were two independent risk factors for nonsurvival (p = 0.04 and p = 0.004, respectively). Patients with a SCORTEN score of 2 or less had no mortality rate in this series. Observed and predicted mortality rates were comparable for patients with SCORTEN scores of 3 or greater. Patients with combined scores of 3 or less had a standardized mortality ratio score of 0.58 (42 percent mortality reduction). Overall, the standardized mortality ratio was 0.89 (11 percent mortality reduction). CONCLUSIONS: Transfer to a burn intensive care unit and initiation of critical care and wound protocols similar to those used for burn patients are recommended for patients with toxic epidermal necrolysis syndrome. Antishear wound care provides an effective alternative wound care approach with equivalent mortality rates.
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Cuidados Críticos/métodos , Síndrome de Stevens-Johnson/terapia , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Unidades de Quemados , Niño , Preescolar , Humanos , Persona de Mediana Edad , Síndrome de Stevens-Johnson/etiología , Heridas y Lesiones/terapiaRESUMEN
BACKGROUND: Toxic epidermal necrolysis (TEN) is a severe and potentially fatal drug reaction characterized by an extensive skin rash with blisters and exfoliation, frequently accompanied by mucositis. The wounds caused by TEN are similar to second-degree burns and severe cases may involve large areas of skin loss. OBJECTIVES: Analysis of our results in patients with TEN and evaluation of the variety of therapeutic interventions that has been studied and suggested in TEN. PATIENTS/METHODS: Retrospective analysis of 19 consecutive patients with TEN treated in our burns centre between 1989 and 2004. RESULTS: Immediate withdrawal of any potentially fatal drug, maximum supportive care, and a restricted and tailored antibiotic, medical and surgical treatment regimen confined mortality to 21%, whereas prognosis scores like APACHE II and SCORTEN predicted mortality of 22 and 30%, respectively. A positive contribution of selective digestive decontamination is suggested but has yet to be established. CONCLUSIONS: Because of a potentially fatal outcome, fast referral of a patient suspected of TEN to a specialized centre (mostly a burns unit or specialized dermatology centre) for expert wound management and tailored comprehensive care is strongly advised and contributes to survival.
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Síndrome de Stevens-Johnson/terapia , Adolescente , Adulto , Anciano , Unidades de Quemados , Niño , Humanos , Persona de Mediana Edad , Países Bajos/epidemiología , Pronóstico , Derivación y Consulta , Estudios Retrospectivos , Síndrome de Stevens-Johnson/mortalidad , Resultado del TratamientoRESUMEN
BACKGROUND: Banana leaf is used in many centers in India during the care of patients with toxic epidermal necrolysis (TEN) and other extensive blistering disorders. Sepsis is an important cause of death in TEN patients and use of banana leaf may be a source of such infection. AIMS: We conducted this study to detect the bacterial flora of the banana leaf and to examine various methods of rendering the leaf aseptic. METHODS: Five pieces of banana leaf, 2 x 2 cm in size, were cultured separately in blood agar as follows: One piece was heated over a flame and one was soaked in boiling water and one was autoclaved. Methylated spirit was applied over one piece and ignited. One piece was placed on the media, 'as is.' The Petri dishes were incubated examined after 48 h. RESULTS: All the pieces except the autoclaved specimen of the leaf grew coagulase-negative staphylococci (CONS) when aseptic precautions were not maintained and aerobic spore bearers when all aseptic measures were subsequently instituted during the procedure. CONCLUSION: We recommend measures to prevent possible transmission of bacterial infection by the leaf. Autoclaved and aseptically handled banana leaves may be used to reduce chance of infection in the treatment of TEN.