Integrating antimicrobial photodynamic therapy into the adjuvant phototherapeutic approach for orofacial manifestations of toxic epidermal necrolysis and Stevens-Johnson syndrome.

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.

Afecciones perianales no infecciosas, no neoplásicas, del adulto / Noninfectious, non-neoplasic adult perianal affections

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)

Stevens-Johnson syndrome and toxic epidermal necrolysis - retrospective review of cases in a high complexity hospital in Brazil.

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.

Stevens-Johnson syndrome and toxic epidermal necrolysis: a retrospective descriptive study.

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.

The Role of Biological Skin Substitutes in Stevens-Johnson Syndrome: Systematic Review.

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.

Necrolisis epidérmica tóxica: un paradigma de enfermedad crítica / Toxic epidermal necrolysis: a paradigm of critical illness

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.

Toxic epidermal necrolysis: a paradigm of critical illness. / Necrolisis epidérmica tóxica: un paradigma de enfermedad crítica.

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.

Abordagem cutânea na necrólise epidérmica tóxica / Cutaneous approach in toxic epidermal necrolysis

Introdução: Necrólise epidérmica tóxica é uma erupção mucocutânea aguda grave, geralmente induzida por medicamentos, associada a alta taxa de morbidade e mortalidade. Os cuidados com as lesões mucosas e cutâneas e a abordagem multidisciplinar são muito importantes para o prognóstico e sequelas futuras. Objetivos: Discutir os principais aspectos dessa síndrome por meio da revisão de literatura, ilustrada por um caso clínico. Métodos: Revisão de literatura utilizando bases de dados on-line PubMed e Scielo. Incluímos artigos em língua inglesa, portuguesa, francesa e espanhola, e ilustração com caso clínico pediátrico. Termos procurados foram "toxic epidermal necrolysis", "Stevens-Johnson overlap", ''necrólise epidérmica tóxica'', ''síndrome Stevens-Johnson''. Resultados: Apresentamos dados para guiar o manejo de pacientes com necrólise epidérmica tóxica para cirurgiões plásticos, pediatras, intensivistas, dermatologistas e emergencistas. O caso tratado teve evolução favorável, sem sequelas cutâneas. Conclusão: O alto nível de suspeição é imprescindível para um diagnóstico e estratificação de risco adequados e instituição precoce de medidas de suporte, e o tratamento deve ser realizado por uma equipe multidisciplinar treinada para reduzir sequelas e mortalidade.

Introduction: Toxic epidermal necrolysis is a severe acute mucocutaneous condition usually induced by drugs associated with a high rate of morbidity and mortality. The care of the mucous lesions and skin and a multidisciplinary approach are very important for the prognosis and future sequelae. Objectives: To discuss the main aspects of this syndrome through a literature review illustrated by a clinical case. Methods: Review of the literature using the PubMed and SciELO online databases was performed. Articles in English, Portuguese, French, and Spanish were included and illustrated with a pediatric clinical case. The keywords used were as follows: "toxic epidermal necrolysis," "Stevens-Johnson overlap," "necrólise epidérmica tóxica," and "síndrome Stevens-Johnson." Results: We presented data to guide the management of patients with toxic epidermal necrolysis for plastic surgeons, pediatricians, intensivists, dermatologists, and emergency physicians. The case treated had a favorable disease course without sequelae. Conclusion: A high level of suspicion is necessary for an adequate diagnosis and risk stratification, and early support measures and treatment should be performed by a multidisciplinary team trained to minimize damage and mortality.

Toxic Epidermal Necrolysis in Recessive Dystrophic Epidermolysis Bullosa following Bone Marrow Transplantation.

A 3-year-old child with recessive dystrophic epidermolysis bullosa treated with bone marrow transplantation subsequently developed body-wide epidermal detachment distinct from his epidermolysis bullosa. Toxic epidermal necrolysis was diagnosed by examination and skin biopsy. Although graft-vs-host disease was considered, he had no features of this diagnosis by laboratory studies or skin biopsy, and he improved without addition of further immune suppressants. Throughout the episode, the patient was maintained on cyclosporine A, a component of his transplant regimen, and also a reported therapy for toxic epidermal necrolysis. He had full recovery. Re-epithelialization occurred in a unique folliculocentric pattern, which we postulate was related to the patient's mesenchymal stem cell infusion, received as an adjunct to his marrow transplantation.

[News in severe clinical adverse drug reactions: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)]. / Actualidades en farmacodermias severas: síndrome de Stevens-Johnson (SSJ) y necrólisis epidérmica tóxica (NET).

Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening conditions associated with significant morbidity and mortality. They are considered to be part of a spectrum of cutaneous drug reactions, differing only by their extent of skin detachment due to keratinocyte apoptosis. Drugs are assumed as the main cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in most cases. The pathophysiology is incompletely understood; however, current pathogenic models involve Fas ligand, granulysin, and cytokines. Diagnosis relies mainly on clinical signs together with the histological analysis, and treatment requires early cessation of the causative drug and supportive care. Of these conditions, herein we will review the advances in clinical, pathogenesis, and management.

[Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in a hospital in Distrito Federal]. / Síndrome de Stevens-Johnson e Necrólise Epidérmica Tóxica em um hospital do Distrito Federal.

This study aimed to analyze demographic and clinical aspects of patients diagnosed with Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), as well as identifying the actions of health professionals for the management of Adverse Drug Reactions (ADR) in a public hospital in Distrito Federal, Brazil. A descriptive and retrospective research was held, with quantitative approach. Data collected from all the records of 22 patients admitted with diagnosed with SJS and TEN, from January 2005 to September 2012. Data were analyzed using descriptive statistics. Of these cases, 9 were diagnosed with NET and 7, with SJS; there were more females (14); aged from 21 to 40 years (10); 21 were cured; the drugs more used were the antiepileptic ones (10). Fragility in clinical registers and in the actions to monitor the cases of ADR in this health service was observed.

[POTENTIALLY FATAL LYELL SYNDROME]. / SINDROME DE LYELL POTENCIALMENTE FATAL.

Toxic epidermal necrolysis or Lyell's syndrome is a rare entity with strong compromise of immune system. It is a hypersensitivity reaction secondary to the use of specific drugs and other triggers. Erythematous lesions of bullous aspect characterize the clinical picture affecting all layers of the skin, scaly and widespread in distribution and progression. Sometimes life threatening, generate a severe inflammatory and systemic reaction with multiple organ dysfunction. Treatment encompasses three objectives: identification and suspension of triggers, general clinical support and therapy to the skin lesions. Immune therapy is controversial and not supported by solid scientific evidence. We report a case that summarizes all aspects of this potentially lethal disease.

[Toxic epidermal necrolysis associated with acute infection by Mycoplasma pneumoniae]. / Necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae.

Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis represent different manifestations of the same spectrum of severe idiosyncratic cutaneous reactions to drugs and to a lesser extent are associated with infectous agents. Among these, Mycoplasma pneumoniae is one of the most frequent. We report the case of a female patient aged 5 years, with a toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection, which begins with a fever accompanied by a generalized rash with involvement of the mucous membranes. IgM serology for Mycoplasma pneumoniae was positive and initial biopsy was compatible with erythema multiforme major. The patient was treated with corticosteroids, intravenous immunoglobulin, plasmapheresis and strict care to prevent superinfection and sequels. After 31 days of hospitalization the patient was discharged from hospital.

Necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae / Toxic epidermal necrolysis associated with acute infection by Mycoplasma pneumoniae

El eritema multiforme, el síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica representan diferentes manifestaciones de un mismo espectro de graves reacciones cutáneas idiosincrásicas a fármacos y, en menor medida, están asociados a agentes infecciosos. De estos últimos, Mycoplasma pneumoniae es uno de los más frecuentes. Se presenta el caso de una niña de 5 años, con una necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae, que comenzó con un cuadro febril acompañado de un exantema generalizado y compromiso de todas las mucosas. Se obtuvo serología IgM positiva para Mycoplasma pneumoniae y una biopsia inicial compatible con eritema multiforme mayor. La paciente fue tratada con corticosteroides, gammaglobulina intravenosa, plasmaféresis y estrictos cuidados para la prevención de sobreinfección y posibles secuelas. Después de 31 días de internación fue dada de alta hospitalaria.

Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis represent different manifestations of the same spectrum of severe idiosyncratic cutaneous reactions to drugs and to a lesser extent are associated with infectous agents. Among these, Mycoplasma pneumoniae is one of the most frequent. We report the case of a female patient aged 5 years, with a toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection, which begins with a fever accompanied by a generalized rash with involvement of the mucous membranes. IgM serology for Mycoplasma pneumoniae was positive and initial biopsy was compatible with erythema multiforme major. The patient was treated with corticosteroids, intravenous immunoglobulin, plasmapheresis and strict care to prevent superinfection and sequels. After 31 days of hospitalization the patient was discharged from hospital.

Necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae / Toxic epidermal necrolysis associated with acute infection by Mycoplasma pneumoniae

El eritema multiforme, el síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica representan diferentes manifestaciones de un mismo espectro de graves reacciones cutáneas idiosincrásicas a fármacos y, en menor medida, están asociados a agentes infecciosos. De estos últimos, Mycoplasma pneumoniae es uno de los más frecuentes. Se presenta el caso de una niña de 5 años, con una necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae, que comenzó con un cuadro febril acompañado de un exantema generalizado y compromiso de todas las mucosas. Se obtuvo serología IgM positiva para Mycoplasma pneumoniae y una biopsia inicial compatible con eritema multiforme mayor. La paciente fue tratada con corticosteroides, gammaglobulina intravenosa, plasmaféresis y estrictos cuidados para la prevención de sobreinfección y posibles secuelas. Después de 31 días de internación fue dada de alta hospitalaria.(AU)

Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis represent different manifestations of the same spectrum of severe idiosyncratic cutaneous reactions to drugs and to a lesser extent are associated with infectous agents. Among these, Mycoplasma pneumoniae is one of the most frequent. We report the case of a female patient aged 5 years, with a toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection, which begins with a fever accompanied by a generalized rash with involvement of the mucous membranes. IgM serology for Mycoplasma pneumoniae was positive and initial biopsy was compatible with erythema multiforme major. The patient was treated with corticosteroids, intravenous immunoglobulin, plasmapheresis and strict care to prevent superinfection and sequels. After 31 days of hospitalization the patient was discharged from hospital.(AU)

Necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae. / [Toxic epidermal necrolysis associated with acute infection by Mycoplasma pneumoniae].

Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis represent different manifestations of the same spectrum of severe idiosyncratic cutaneous reactions to drugs and to a lesser extent are associated with infectous agents. Among these, Mycoplasma pneumoniae is one of the most frequent. We report the case of a female patient aged 5 years, with a toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection, which begins with a fever accompanied by a generalized rash with involvement of the mucous membranes. IgM serology for Mycoplasma pneumoniae was positive and initial biopsy was compatible with erythema multiforme major. The patient was treated with corticosteroids, intravenous immunoglobulin, plasmapheresis and strict care to prevent superinfection and sequels. After 31 days of hospitalization the patient was discharged from hospital.

Steven Johnson Syndrome / Síndrome de Steven Johnson

Ulcerative vesiculobullous disorders are common in Dermatology and Oral Medicine. Diagnosis of these conditions is pretty effortless if they give a classic appearance as described in the literature. Steven Johnson syndrome is one such condition which is a type of erythema multiforme. It is a disorder involving mucous membrane, skin and even the multiple organs in severe form. Multiple etiologies persist so be acquainted with the cause and prohibit the root is crucial. But still drugs are considered to be common cause. Early management is vital as complications are serious for this condition even leading to death. We hereby report a typical case with classic appearance of Steven Johnson syndrome.

Doenças ulcerativas vésicobolhosas são comuns em Dermatologia e Medicina Oral. O diagnóstico destas condições é bastante fácil se apresentar uma aparência clássica, tal como descrito na literatura. Steven Johnson é um tipo de eritema multiforme. É uma desordem envolvendo as mucosas, pele e até múltiplos órgãos de forma grave. Múltiplas etiologias são apontadas como causa. Mas ainda os medicamentos são considerados como causas comuns. O tratamento precoce é vital, pois complicações são graves para essa condição, podendo levar à morte. Vimos por meio deste relatar um caso típico, com aparência clássica de Síndrome de Steven Johnson.