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.
Assuntos
Fotoquimioterapia , Fármacos Fotossensibilizantes , Síndrome de Stevens-Johnson , Humanos , Terapia Combinada , Terapia com Luz de Baixa Intensidade/métodos , Fotoquimioterapia/métodos , Fármacos Fotossensibilizantes/uso terapêutico , Síndrome de Stevens-Johnson/tratamento farmacológico , Síndrome de Stevens-Johnson/terapiaRESUMO
Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)
Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)
Assuntos
Humanos , Adulto , Doenças do Ânus/diagnóstico , Doenças do Ânus/terapia , Equipe de Assistência ao Paciente , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/terapia , Dermatite Herpetiforme/diagnóstico , Dermatite Herpetiforme/terapia , Penfigoide Mucomembranoso Benigno/diagnóstico , Penfigoide Mucomembranoso Benigno/terapia , Pênfigo/diagnóstico , Pênfigo/terapia , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/terapia , Pênfigo Familiar Benigno/diagnóstico , Pênfigo Familiar Benigno/terapia , Dermatite de Contato/diagnóstico , Dermatite de Contato/terapiaRESUMO
BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare conditions characterized by epidermal necrosis, mostly caused by drugs. Despite the rarity of these conditions, morbidity and mortality are high (even in previously healthy patients), and they may be associated with permanent sequelae. METHODS: A retrospective study conducted at a quaternary hospital in Brazil in a period of 10 years. RESULTS: The sample was composed by 41 patients with SJS, SJS/TEN, and TEN confirmed by skin biopsy. Antibiotics and anticonvulsants were the most frequently implied drug classes, and phenytoin was the most important individual culprit drug. In this study, 12.2% of the patients had sequelae, being ophthalmological lesions the most common and one case of a newly described hearing loss. The mortality rate was 16.7% in patients with TEN. CONCLUSIONS: This study describes the largest Latin American case series of SJS and TEN with the diagnosis proven by skin biopsy and adds important data regarding the profile of the disease in Brazil. It also describes a novel sequelae of hearing loss.
Assuntos
Antibacterianos/efeitos adversos , Anticonvulsivantes/efeitos adversos , Síndrome de Stevens-Johnson/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Brasil/epidemiologia , Criança , Feminino , Perda Auditiva/etiologia , Hospitais , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/mortalidade , Síndrome de Stevens-Johnson/terapia , Triquíase/etiologia , Estreitamento Uretral/etiologia , Adulto JovemRESUMO
BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and potentially life-threatening mucocutaneous reactions. Given their rarity, limited cohort studies have been done. The aim of this study is to evaluate and compare the demographics, etiology, management, clinical and laboratory characteristics, complications, and outcome of SJS/TEN patients seen by the inpatient dermatology service at the University of Puerto Rico. METHODS: A retrospective review of 30 cases with identified diagnosis of SJS, overlap SJS/TEN, or TEN who were consulted to the Dermatology Department of the University of Puerto Rico from 2006 to 2017. RESULTS: A total of 24 adult and six pediatric cases were reviewed. Females were predominant with a female to male ratio of 1.3 : 1. The most frequent offending drugs identified were antibiotics (56.7%), anticonvulsants (23.3%), and nonsteroidal anti-inflammatory drugs (NSAIDs) (16.7%) with the most frequent antibiotic identified being trimethoprim/sulfamethoxazole (23.3%). Seventy percent of patients experienced at least one complication, most often of infectious etiology (80.1%). During hospital course, 73% received pharmacologic therapy (23% received IVIG alone, 17% received steroids alone, and 33% both) versus 27% which received only supportive care. Mortality rate in this study was 13.8%. When comparing SCORTEN at day one of admission, deceased cases had a mean SCORTEN at day 1 of 4.0, while survivors had an average of 1.54 (P < 0.001). CONCLUSION: Antibiotics followed by anticonvulsants were the most frequently offending drugs identified within this study.
Assuntos
Antibacterianos/efeitos adversos , Anti-Inflamatórios não Esteroides/efeitos adversos , Anticonvulsivantes/efeitos adversos , Síndrome de Stevens-Johnson/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Mortalidade Hospitalar , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Pessoa de Meia-Idade , Cuidados Paliativos/métodos , Porto Rico , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/mortalidade , Síndrome de Stevens-Johnson/terapia , Adulto JovemRESUMO
Stevens-Johnson syndrome (SJS) is a disease that causes skin exfoliative lesions, characterized by fever, necrosis, and epidermal detachment. Biological skin substitutes may be considered interesting options for the treatment of this disease. This study aims at identifying in the literature the evidence on the current role of these biomaterials in the treatment of SJS and its related diseases. A systematic review with a search period between 2003 and 2017 was carried out, consulting the Lilacs, BVS, and PubMed databases. The quality of the included studies was evaluated by the Oxford Center for Evidence-Based Medicine Classification, for evaluating levels of evidence from the scientific publications. Ninety-five publications were found, and after applying inclusion and exclusion criteria, they resulted in 9 articles. In total, 20 patients with 73.87% average of body surface affected received SJS skin treatment with some biological substitutes, among them are cutaneous allograft, amnion, and xenograft. Mortality rate was 10%, and in these situations, literature indicates mortality rates ranging from 25% to 70%. Effectiveness of the use of biological dressings may be a possible explanation for this finding. Findings indicate the use of these biomaterials may favor reepithelialization, reduce water loss, decrease the chance of infection, and, consequently, improve the survival of patients with SJS and toxic epidermal necrolysis. Biological skin substitutes have characteristics that make them very promising in the topical treatment of these wounds, but their use remains very restricted in the treatment of SJS.
Assuntos
Pele Artificial/normas , Síndrome de Stevens-Johnson/terapia , Fenômenos Biológicos , HumanosRESUMO
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.
Assuntos
Humanos , Síndrome de Stevens-Johnson/fisiopatologia , Estado Terminal , Imunossupressores/uso terapêutico , Imunoglobulina G/uso terapêutico , Taxa de Sobrevida , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/terapia , Ciclosporina/uso terapêutico , Progressão da DoençaRESUMO
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.