Fotobiomodulação na síndrome de stevens-johnson: relato de caso / Photobiomodulation for stevens-johnson syndrome: case report

Introdução: A Síndrome Stevens-Johnson (SSJ) é uma doença causada por hipersensibilidade a imunocomplexos e pode ser desencadeada por distintos fármacos, dentre eles a fenitoína. Devido sua complexidade e raridade, ainda nãohá consenso de tratamento padrão ouro, porém sabese da necessidade da atuação multidisciplinar. Para os cuidados com as feridas, pode-se citar os curativo se a fotobiomodulação (FBM). Objetivo: Relatar o uso da FBM como terapia complementar em um caso de SSJ no Hospital Universitário Regional dos Campos Gerais (HU-UEPG). Métodos: Paciente sexo feminino, 26 anos, deu entrada na unidade de terapia intensiva (UTI) com diagnóstico de SSJ secundária ao uso de fenitonína, escore de SCORTEN 1, com área sem epitélio íntegro 10- 30% e área acometida por lesões de 94,5%, poupando apenas o couro cabeludo. Foi abordada e tratada por uma equipe multidisciplinar e solicitado vaga em centro de especializado em queimados. No sétimo dia de UTI foi iniciado tratamento com FBM, 2 J por ponto, distância entre pontos de 2cm, comprimento onda vermelho (660nm), nas feridas que não apresentavam secreção, foram cinco sessões com intervalo de três dias entre a terceira e a quarta. Resultados: A paciente apresentou melhora visível das lesões cutâneas e recebeu alta hospitalar 5 dias após cessação da FBM. Conclusão: O uso da FBM pode ser efetiva no tratamento complementar da fase aguda SSJ desencadeada por fenitoína.

Introduction: Stevens-Johnson Syndrome (SJS) is a disease caused by hypersensitivity to immune complexes and can be triggered by different drugs, including phenytoin. Due to its complexity and rarity, there is still no consensus on gold standard treatment, but the need for multidisciplinary action is known. For wound care, dressings and photobiomodulation (PBM) can be mentioned. Objective: This study is to report the use of PBM as complementary therapy in a case of SJS at Hospital Universitário Regional dos Campos Gerais (HU-UEPG). Methods: A 26-year-old female patient was admitted to the intensive care unit (ICU) diagnosed with SJS secondary to the use of phenytoin, SCORTEN score 1, with an area without intact epithelium 10-30% and an area affected by injuries of 94.5 %, saving only the scalp. She was approached and treated by a multidisciplinary team which requested a place in a specialized burn center. On the seventh day of ICU, treatment with PBM, 2J per point was started, distance between points of 2cm, red wave length (660nm), in wounds that did not present secretion, with a total of five sessions with an interval of three days between the third and fourth. Results: The patient showed a visible improvement of skin lesions and was discharged from hospital 5 days after cessation of PBM. Conclusion: Use of PBM can be effective in complementary treatment of acute SJS phase triggered by phenytoin.

Vasculitis por reacción de hipersensibilidad tipo III / Vasculitis for type III hypersensitivity reaction

Los procesos inmunitarios son utilizados por el organismo para defenderse de la agresión de agentes infecciosos; no obstante, en ciertos casos, el organismo reacciona de forma inapropiada o excesiva ocasionando diversos tipos de daño tisular. Estas situaciones, que conocemos como hipersensibilidad, pueden tener aspectos positivos o negativos al poder causar ellos mismos la enfermedad. Se presenta el caso de una niña de 14 años de edad, que acude al Hospital Pediátrico Docente William Soler después de varios ingresos en otros centros de salud, donde se planteó el diagnóstico de un pie de madura. Después de varias investigaciones y con el antecedente de alergia a diferentes medicamentos, los cuadros de amigdalitis a repetición, los datos del laboratorio y la clínica que presentaba la paciente, se estableció el diagnóstico de una vasculitis por reacción de hipersensibilidad tipo III. Por las características tan atípicas del cuadro clínico de esta paciente y la dificultad para llegar a un diagnóstico es importante la presentación de este caso(AU)

The immune processes are used by the body to defend against the aggression of infectious agents; however, in certain cases, the body reacts inappropriately or excessively causing various types of tissue damage. These situations, which we know as hypersensitivity, can have positive or negative aspects by being able to cause the disease themselves. We present the case of a 14-year-old girl who attended the William Soler Pediatric Teaching Hospital after several admissions to other health centers, where the diagnosis of a mature foot was raised. After several investigations and with the history of allergy to different drugs, the recurrent tonsillitis symptoms, the laboratory data and the clinic presented by the patient, the diagnosis of a vasculitis due to type III hypersensitivity reaction was established. Because of the atypical characteristics of this patient's clinical picture and the difficulty in reaching a diagnosis, the presentation of this case is important(AU)

Lupus-like Glomerulonephritis Associated with Extranodal Marginal Zone B-cell Lymphoma of MALT / 대한내과학회지

Lupus-like glomerulonephritis is an immune complex disease with features of lupus nephritis in the absence of systemic lupus erythematosus (SLE). We report a 49-year-old man diagnosed with lupus-like glomerulonephritis associated with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). He was admitted to the hospital for edema. At admission, the serum creatinine was 2.2 mg/dL and the urine protein level was 3.9 mg/day. A renal biopsy showed features of lupus nephritis with no clinical or serological evidence of SLE. Extranodal marginal zone B-cell lymphoma of MALT was discovered concurrently. After successful chemotherapy, the lupus-like glomerulonephritis and lymphoma entered complete remission.

Reacciones por lepra en un centro de referencia nacional en Colombia / Leprosy reactions in a Colombian national reference centre

Introducción. Colombia es el país de América con mayor proporción de casos nuevos de lepra con discapacidad grave. Para disminuir tal discapacidad se requiere el control de las reacciones, principal causa del daño neural en esta enfermedad. Objetivo. Describir las características clínicas y epidemiológicas y el tratamiento de los pacientes con reacciones de tipo 1 y 2 que consultaron al Centro Dermatológico Federico Lleras Acosta. Materiales y métodos. Se trata de un estudio descriptivo que incluyó la población de pacientes con diagnóstico clínico de reacciones de tipo 1 y de tipo 2 por lepra, que acudieron al centro entre los años 2003 y 2009. Resultados. Se estudiaron 96 reacciones, 35 del tipo 1 y 61 del tipo 2. El 75 % de los pacientes provenía de los departamentos de Tolima, Cundinamarca, Santander y Boyacá. El 56 % de las reacciones de tipo 1 se presentaron antes de iniciar la poliquimioterapia para la lepra; el dermatólogo tratante consideró que las reacciones que se presentaron después de suspender la poliquimioterapia eran recaídas. El 94 % de las reacciones de tipo 1 se trataron con corticoides orales. El 97 % de los pacientes con reacciones de tipo 2 presentaron eritema nudoso, y todos se trataron con talidomida. Conclusiones.La clínica de la reacción de tipo 1 puede orientar al diagnóstico de la lepra en un paciente sin el antecedente de esta enfermedad (56 %). La reacción de tipo 1 que se inicia después de suspender la poliquimioterapia para la lepra, podría ser una manifestación de recaída de la enfermedad. La reacción de tipo 2 es más frecuente en hombres, con una relación hombre a mujer de 4:1. El 97 % de los pacientes con reacción de tipo 2 presentó eritema nudoso.

Introduction: Colombia is the country in America with the highest proportion of new cases leprosy with severe disability. To decrease such disability it is necessary to control these reactions, the main cause of nerve damage in leprosy. Objective: To describe the clinical and epidemiological characteristics and the treatment of patients with type 1 and 2 leprosy reactions who consulted the Centro Dermatológico Federico Lleras Acosta. Materials and methods: It is a descriptive study which included patients with clinical diagnoses of type 1 and 2 reactions who were seen in the center between 2003 and 2009. The town of origin of the patients, their age, clinical features and treatments were analysed. Results: We studied 96 reactions in 87 patients, 35 type 1 and 61 type 2 reactions; 75% of the patients came from the departments of Tolima, Cundinamarca, Santander and Boyacá; 77% of type 1 reaction occurred before the beginning of multidrug therapy for leprosy. The reactions that started after stopping the multidrug therapy were considered as a leprosy relapse. Conclusions: Correct identification of type 1 reaction by the general practitioner will allow the diagnosis of leprosy in a large percentage of patients. The type 1 reaction that begins after stopping the leprosy multidrug therapy may be a manifestation of a relapse of the disease.

O Teste do Fator Antinúcleo (FAN) na prática clínica: quando solicitar e como interpretar / The antinuclear factor (ANF) test in clinical practice: when to apply and how to interpret

O teste do Fator Antinúcleo (FAN) é um importante teste para triagem de doenças autoimunes, porém outras condições clínicas alteram esse exame. Ademais, poucos clínicos e generalistas sabem avaliar o seu resultado e, em consequência disso, revisamos como o teste deve ser usado na prática clínica.

The Test for Antinuclear Antibodies (ANA) is a very important factor in the evaluation of autoimmune diseases, however the test can be positive in other conditions in clinical practice, furthermore few generalists and physicians know how to use the results of the test, thereby we reviewed how to correctly use the test in everyday clinical practice.

Enfermedad del suero / Serum sickness

La enfermedad del suero es una enfermedad alérgica rara que se produce por la administración de material antigénico exógeno. Históricamente causada por suero heterólogo; corresponde a una reacción de hipersensibilidad tipo III mediada por depósitos de complejos inmunes circulantes en los pequeños vasos sanguíneos, la cual induce la activación del complemento y subsecuente inflamación. Las características clínicas son fiebre, erupción cutánea, artralgias y linfadenopatías, pudiendo llegar a producir glomerulonefritis o compromiso de otro órgano. Presentamos el caso de un paciente que desarrolló enfermedad del suero posterior a la administración de suero antiofídico.

Serum sickness is a rare allergic disease, produced by the administration of exogenous antigenic material. Historically caused by heterologous serum, it corresponds to a type III hypersensitivity reaction mediated by deposits of circulating immune complexes in small blood vessels, which induces complement activation and subsequent inflammation. Clinical features are fever, rash, arthralgia and lymphadenopathy; this pathology may lead to glomerulonephritis or other organ involvement. We report a patient who developed serum sickness after antivenom administration.

Crescentic glomerulonephritis: A clinical and histomorphological analysis of 46 cases.

Background: Crescentic glomerulonephritis (CrGN), defined as crescents involving more than 50% of the glomeruli, includes pauci-immune, immune complex-mediated and anti-glomerular basement membrane disease. Objectives: The present study was aimed at evaluating the various clinical, biochemical and histological parameters in CrGN with respect to these categories and clinical outcome. Materials and Methods: Renal biopsies diagnosed as CrGN between Jan 2008 and Feb 2010 were included. Clinical and laboratory parameters were retrieved along with the therapeutic approach and clinical outcome, wherever available. Renal biopsy slides were evaluated for various glomerular, tubulo-interstitial and arteriolar features. Appropriate statistical tests were applied for significance. Results: A total of 46 cases of CrGN were included; majority (71.7%) of cases were pauci-immune (PI) while 28.3% were immune complex-mediated (IC). Among clinical features, gender ratio was significantly different between PI and IC groups (P = 0.006). The various histological parameters, including proportion of cellular crescents, tuft necrosis and Bowman's capsule rupture, were similar in both the groups. Four unusual associations, including idiopathic membranoproliferative glomerulonephritis (MPGN), multibacillary leprosy, acute lymphoblastic leukemia and C1q nephropathy were detected. Adequate follow-up information was available in 21 (46%) of the patients. Of these, 11 (52.4%) were dialysis-dependent at the last follow-up. Adult patients required renal replacement therapy more frequently than pediatric cases (P = 0.05). Presence of arteriolar fibrinoid necrosis also showed association with poor clinical outcome (P = 0.05). Conclusions: Crescentic glomerulonephritis remains one of the main causes of acute renal failure with histological diagnosis. Immunohistologic examination is essential for accurate classification into one of the three categories. This condition should be considered in rare causal associations like leprosy or MPGN with renal failure, to allow for timely performed renal biopsy and appropriate aggressive therapy.

Renal involvement in patients with hepatitis c virus infection

Hepatitis C virus [HCV] infection is a hepatotropic virus causing a variety of extrahepatic immunological manifestations and is a risk factor of a variety of extrahepatic diseases, such as mixed cryoglobulinemia and membranoproliferative glomerulonephritis [MPGN], which is the most common glomerulonephritis. The aim of this study was to evaluate renal involvement in HCV-infected patients. A total of 300 randomly-selected HCV antibody-positive outpatients at the HCV clinic of Shariati hospital were enrolled. Serum creatinine was measured and glomerular filtration rate was estimated accordingly. Urine proteinuria was measured in 24-hour urine samples. The patients were 249 men [83.2%] and 51 women [16.8%] with a mean age of 37.8 +/- 11.7 years [range, 18 to 70 years]. Proteinuria was found in 12 HCV antibody-positive adults [4%], 1 of whom underwent biopsy. He was a 55-year-old man with a 4-month history of facial and lower extremities edema and 3-g proteinuria with a normal kidney function [glomerular filtration rate, 85 mL/min] and normocomplementemia. Kidney biopsy specimens showed MPGN. The frequency of low glomerular filtration rate was 0.7% [2 patients] in the HCV antibody-positive adults. There was no significant relationship between HCV seropositivity and low glomerular filtration rate. Our observations showed renal involvement in HCV antibody-positive patients. Among immune complex glomerular kidney diseases, MPGN without cryoglobulins is thought to be the most common in these patients

[ study of patients admitted with serum sickness in Mofid Children Hospital from [April 2005 to September 2007]]

Serum sickness is a type III hypersensitivity reaction mediated by immune complex deposition with subsequent complement activation, small vessel vasculitis, and tissue inflammation. Disease is caused by exposure to foreign proteins, equine diphtheria antitoxin. However, sporadic cases of serum sickness from non-protein antibiotics such as penicillins continue to occur. A retrospective chart review was conducted in 28 children who were admitted because of serum sickness in Mofid Children Hospital since April 2005 to September 2007. Of 28 cases included in our study, 17 were male and 11 female. 24 of the 28 cases [86%] aged less than five years old. In 25 of the 28 patients [89%], the onset of symptoms occurred 1-3 weeks after administration of the inciting antigen, in two patients less than one week, and in one patient more than three weeks. Associated symptoms included arthritis 36%, subjective fever 75%, arthralgia 85%, skin rash and angioedema 100% were observed during admission. Furazolidone was the most common cause of disease in this study. The incidence of serum sickness is decreasing as a result of public health vaccination programs that have decreased the need for specific antitoxins. However, currently, non-protein drugs such as furazolidone are the most common causes of serum sickness-like reactions

Relationship between Brucella immunocomplex and glomerulopathies

Brucellosis is a zoonotic disease with a very wide spectrum of clinical findings. Brucellosis is about 10 times more prevalent in patients with renal failure [dialysis patients] compared to population background. Precipitation of immunocomplexes produced by brucellosis is important in causing glomerulonephritis. Because the hallmark of glomerular diseases is abnormal protein loss in the urine we have decided to study proteinuria in brucellosis immunocomplex. The aim of this study was to evaluate probable relationship between brucellosis and glomerulonephritis. This cross sectional study, performed on 200 patients with a history of the disease for about 1 year, diagnosed as having chronic brucellosis. The diagnosis was confirmed in 150 patients and by applying Brucella Standard Agglutination Test [SAT] and 2-mercaptoethanol test [2-ME]. Titers of IgG/IgM and IgG/IgA in two groups of "with proteinuria" and "without proteinuria" was measured. Both SAT and 2-ME tests test demonstrated that proteinuria increases with rising antibodies titers. In SAT, titer 1/160 was the most frequent, observed in 44% of the patients. In 2-ME test 1/40 titer was observed in 44% of the patients and was the most frequent. Our results clearly demonstrated that in both chronic and acute patients, proteinuria increases with rising IgG/IgA and IgM/IgG titers. Therefore brucellosis can cause nephropathy but chronic or untreated brucellosis is more important because it can permanently damage kidney

A case of membranous nephropathy in a patient with Graves' disease / 대한내과학회지

Membranous nephropathy is one of the most common causes of the nephrotic syndrome in adults. Membranous nephropathy is known as a disease associated with many other disorders and the presumed etiology of the disease is a deposition of circulating immune complexes. But, it has rarely been reported in association with autoimmune thyroiditis. We report a case of membranous nephropathy associated with Graves' disease and review the literature regarding this disease entity.

Use of complement receptor 1 (CD35) assay in the diagnosis and prognosis of immune complex mediated glomerulopathies.

Complement Receptor 1 (CR1) is a polymorphic glycoprotein expressed on erythrocytes, leukocytes and glomerular podocytes and has a major role in immune complex processing. In addition, it regulates the complement cascade activation by preventing formation of classical and alternative pathway convertases and by acting as a cofactor for Factor I mediated cleavage of C3. In this study, we have examined the expression of erythrocyte CR1 (E-CR1) and glomerular CR1 (G-CR1) in different kinds of nephropathies using ELISA and immunofluorescence microscopy to understand their role in immune complex (IC) mediated renal diseases. E-CR1 was significantly reduced in all categories of lupus nephritis in comparison to normal subjects and non-IC renal diseases. However, other IC mediated diseases like IgA nephropathy and membranoproliferative glomerulonephritis had normal E-CR1 levels. G-CR1 showed distinct differences between IC and non-IC mediated diseases. G-CR1 was virtually absent in lupus kidneys. In other IC mediated diseases, there was a correlation of G-CR1 expression to the IC and complement fragment deposition. G-CR1 serves as a useful diagnostic marker for IC mediated diseases while E-CR1 is useful as a prognostic marker to monitor the course of disease after the treatment has initiated.

Rheumatic disorders in virus-C hepatitis patients

To study the incidence of rheumatologic disorders in chronic hepatitis C infection. Fifty patients suffering from chronic hepatitis C [Positive HCV antibody and PCR] of more than six months duration were included in the study. Another group of twenty-five patients with rheumatic manifestations free of HCV served as a control group. They were identical in gender and age. Among the HCV patients group cryoglobulinaemia was detected in 29 cases [58%], antinuclear antibodies in 1 0 cases [20%] rheumatoid factor in 19 cases [38%], hypocomplementaemia in 20 cases [40%], myalgia in 24 cases [48%], arthralgia in 26 cases [52%] arthritis in 18 cases [36%] enthesitis in 39 cases [78%], fatigability in 46 cases [92%], Raynaud's Phenomenon in 14 cases [28%], xerophthaimia in 1 0 cases [20%], rash in 29 cases [58%], carpal tunnel syndrome in 6 cases [1 2%] and R A in 6 cases [1 2%]. HCV may play a role in the pathogenosis of these auto immune phenomena. This hypothesis is mainly based on the lymphotropism of HCV that can be responsible for a chronic activation of both T and B lymphocytes, leading to complex immune system disorders. But more studies are required to define the extent of this role

A case of systemic lupus erythematosus with chylothorax, chronic interstitial cystitis and protein-losing enteropathy / 대한내과학회지

Systemic lupus erythematosus(SLE) is the prototypic immune complex disease which involves various organs. Chronic interstitial cystitis and protein-losing enteropathy are uncommon manifestations of SLE and have good response to steroid. Only one case of SLE associated with chronic interstitial cystitis and protein-losing enteropathy has been reported which was resistant to steroid treatment but dramatically responded to cyclophosphamide. We describe a case of a female patient who developed systemic lupus erythematosus associated with chylothorax, interstitial cystitis and protein-losing enteropathy with literature review. This case was initially resistant to steroid but with the addition of cyclophosphamide, chylothorax, chronic interstitial cystitis and protein-losing enteropathy markedly improved. This is the first case of SLE that developed chylothorax as well as chronic interstitial cystitis and protein-losing enteropathy.

A Study on Comparison of the Metabolites Related with Working Methods and Immunity of the Toluene diisocyanate-Exposed Workers / 대한산업의학회지

Following recent advanced industrialization. the amount of polyurethane to use as thermal insulating materials, upholstery mattresses and packing materials in automotive and furniture industry is increasing world-widely, and the number of polyurethane-producing worker will be increased. Because the numerous organic solvents are used in polyurethane-producing factory, the workers in this work site is exposed to many organic solvents. Of the organic solvents. Toluene Diisocyanate(TDI) has many hazardous effects to human. The effects of TDI on human are the irritation to respiratory mucosa and gastrointestinal symptoms. Conjunctival irritation, dermal inflammation (redness, pain, vesicular formation) and gastrointestinal symptom(nausea, vomiting, abdominal pain) are reported just after short-term exposure of TDI. TDI is known to give rise to bronchial asthma, as the immune disorder. And because of strongly volatile characteristics of TDI, it is suggested as a more injurious material to human health, especially human immune system, than other organic solvents. Bronchial asthma inducing mechanism of TDI is not clearly known, but on the analogy of TDI-induced symptoms and recent studies, early-onset asthma is type I hypersensitivity reaction mediated by immunoglobulin E(IgE), and late-onset asthma is maybe type III hypersensitivity reaction by circulating IgG. And we know that the complicated human immune function is likely to move in such that mechanisms, there are not studies on immune indices evaluating the bronchial asthma-related immune function. The evaluation of change patterns of humoral immunity including IgE and IgG and cellular immunity including T-helper cell, T-suppressor cell and T-cytotoxic cell will be helpful to evaluate exposure degrees and prognosis in TDI-exposed workers. Because TDA(toluene diamine) as a biological exposure index of TDI becomes the focus of interest, we know that a study on the correlation between urinary TDA and air TDI and immunological indices will make a contribution to biological effect monitoring indicies. We examined human immunity indicators such as WBC. %Lymph (percentile of Lymphocyte in WBC). %T-cell(percentile of T-lymphocyte in total lymphocyte). CD4, CD8, C3, C4, IgA, IgG, IgM, IgE in peripheral blood to evaluate the health hazard of the TDI-exposed workers. And we examined TDA to evaluate correlation between exposure and effect. Total 90 subjects was selected, 45 workers who worked in the polyurethane-producing factories as an exposed group, and 45 cases who were office workers(10 cases), other blue collors(27 cases), and medical college students(8 cases) as a control group. And the results were as follows ; 1. The logarithm of IgE -Log10(IgE)+/-SD- in peripheral blood of a exposed group was significantly higher than a control group, 2 22+/-.62 in case group compared with 1.98+/-.53 in control group.(p0.05). 3. WBC, %Lymph, %T-cell, C3, C4, CD4, CD8, CD4/CDB ratio and IgG in case group were 6,391.1 ea/ml, 37.53%, 59.54%, 76.68 mg/dl, 30.54 mg/dl, 0.76x10(9) ea/L, 0.63x10(9) ea/L, 1.39, and 1606.29 mg/dl, respectively, and 6,974.7 ea/ml, 35.12%, 59.64%, 71.95 mg/dl, 33.94 mg/dl, 0.80x109 ea/L, 0.61x10(9) ea/L, 1.39, and 1581.51 mg/dl in control group. There was no statistical sinificance between two groups. (p>0.05) 4. In the comparison of each other companies, average of individual urinary TDA in polyurethane paint manufacturing companies is higher than that of polyurethane sponge foaming companies. And, the concentration of 2,6-TDA which is a metabolite of well-vaporized 2,6-TDI is higher than that of 2,4-TDA in the polyurethane sponge foaming companies. But, the concentration of 2,4-TDA which is a metabolite of illvaporized but well skin-absorbed 2,4-TDI is higher in polyurethane paint manufactures. 5. There were no statistical significance in the correlations between individual urinary TDA and immunologic indices.

Clearance de imunocomplexos: papel do complemento e dos polimorfonucleares neutrófilos / Clearance of immune complexes: role of complement and polymorphonuclear neutrophils

O sistema complemento desempenha um papel importante no transporte e eliminaçäo de imunocomplexos (IC) circulantes. No entanto, existem circunstâncias em que os mecanismos envolvidos näo säo eficientes, favorecendo a deposiçäo dos IC em órgäos específicos. Tais complexos depositados estimulam a fagocitose pelos polimorfonucleares neutrófilos, com conseqüente produçäo de radicais de oxigênio e liberaçäo de enzimas lisossomais, e contribuem para a lesäo tecidual local. Nesta revisäo, damos ênfase ao papel do complemento, bem como dos neutrófilos, na patogênese das doenças por imunocomplexos.