Ocular sarcoidosis masked by positive IgM for toxoplasmosis.

We report a case of ocular sarcoidosis with positive immunoglobulin (Ig) M and IgG serology for toxoplasmosis. The patient was a young female with red painful eyes, bilateral eyelid edema, and panuveitis with periphlebitis. In laboratory testing, she was IgM and IgG positive for toxoplasmosis and anergic in the tuberculin test. Topical treatment for anterior uveitis and oral antibiotics for toxoplasmosis were started, without improvement. Orbit tomography showed increased lacrimal glands bilaterally, and chest X-ray radiographic findings were consistent with pulmonary sarcoidosis, which supported the presumed ocular sarcoidosis diagnosis. The patient was treated with oral prednisone and methotrexate without antibiotics. She showed clinical and vision improvement without recurrences during the 1-year follow-up. Ocular sarcoidosis is an important differential diagnosis requiring careful anamnesis and ophthalmological examinations. Ancillary tests, such as X-ray radiography, tomography, and clinical and laboratory evaluations may help rule out other causes. Treatment mainly consists of corticosteroids and immunosuppression.

Ocular sarcoidosis masked by positive IgM for toxoplasmosis / Sarcoidose ocular mascarada por IgM positivo para toxoplasmose

ABSTRACT We report a case of ocular sarcoidosis with positive immunoglobulin (Ig) M and IgG serology for toxoplasmosis. The patient was a young female with red painful eyes, bilateral eyelid edema, and panuveitis with periphlebitis. In laboratory testing, she was IgM and IgG positive for toxoplasmosis and anergic in the tuberculin test. Topical treatment for anterior uveitis and oral antibiotics for toxoplasmosis were started, without improvement. Orbit tomography showed increased lacrimal glands bilaterally, and chest X-ray radiographic findings were consistent with pulmonary sarcoidosis, which supported the presumed ocular sarcoidosis diagnosis. The patient was treated with oral prednisone and methotrexate without antibiotics. She showed clinical and vision improvement without recurrences during the 1-year follow-up. Ocular sarcoidosis is an important differential diagnosis requiring careful anamnesis and ophthalmological examinations. Ancillary tests, such as X-ray radiography, tomography, and clinical and laboratory evaluations may help rule out other causes. Treatment mainly consists of corticosteroids and immunosuppression.

RESUMO Relatamos um caso de sarcoidose ocular com IgM e IgG positivos para toxoplasmose. Mulher jovem com quadro ocular bilateral de dor, vermelhidão, edema palpebral e panuveíte com periflebite. Os testes laboratoriais mostraram IgM e IgG positivos para toxoplasmose, teste tuberculínico anérgico. Realizou-se tratamento tópico da uveíte anterior e antibióticos orais para toxoplasmose, sem melhora. Tomografia de órbita mostrou aumento das glândulas lacrimais bilaterais e radiografia de tórax foi consistente com sarcoidose pulmonar, auxiliando no diagnóstico de sarcoidose ocular presumida. Iniciou-se prednisona e metotrexato orais, sem antibióticos, com melhora clínica e oftalmológica, sem recidivas em 1 ano de follow-up. Sarcoidose ocular é um importante diagnóstico diferencial que exige anamnese e exame oftalmológico cuidadosos. Exames complementares, como raio-X, tomografia computadorizada e avaliação clínica e laboratorial ajudam na avaliação e exclusão de outras causas. O tratamento consiste principalmente no uso de corticosteróide e imunossupressores.

Coroidopatia no lúpus eritematoso sistêmico / Choroidopathy in systemic lupus erythematosus

Resumo O Lúpus Eritematoso sistêmico (LES) é uma doença autoimune que pode afetar o olho de diversas formas. A coroidopatia lúpica é rara e apresenta-se com descolamento seroso de retina, descolamento do epitélio pigmentar da retina (EPR) e epiteliopatia pigmentar. A maioria dos casos está associada à atividade sistêmica da doença, podendo ser considerada um fator de gravidade e necessidade de imunossupressão intensa. Geralmente apresenta bom prognóstico visual com o tratamento adequado do LES, apesar de alguns casos evoluírem com danos irreversíveis na retina externa e EPR. Descrevemos um caso de coroidopatia secundaria ao LES com atividade multisistêmica com boa evolução após tratamento clínico com imunossupressão sistêmica.

Abstract Systemic lupus erythematosus (SLE) is an autoimmune disease in which can affect the eye in different ways. Lupus choroidopathy is rare and include retinal pigment epithelium (RPE) detachment and/or serous retinal detachment and pigment epitheliopathy. Most cases are associated with systemic disease activity and can be considered a factor of gravity and need for intense immunosuppression. Usually has good visual prognosis with proper treatment of SLE, although some cases may have irreversible damage to the outer retina and RPE. We describe a case of choroidopathy secondary to SLE during its multisystem activity with good clinical outcome after treatment with systemic immunosuppression.

DRESS syndrome in ophthalmic patients.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal adverse drug reaction associated with skin rash, fever, eosinophilia, and multiple organ injury. A number of pharmacological agents are known to cause DRESS syndrome such as allopurinol, anticonvulsants, vancomycin, trimethoprime-sulfamethoxazole, and pyrimethamine-sulfadiazine. Here, we describe two patients who developed DRESS syndrome during ocular treatment. The first case was being treated for late postoperative endophthalmitis with topical antibiotics, intravenous cephalothin, meropenem, and intravitreal injection of vancomycin and ceftazidime before symptoms developed. We were unable to identify the causal drug owing to the large number of medications concurrently administered. The second case presented with DRESS syndrome symptoms during ocular toxoplasmosis treatment. In this case, a clearer association with pyrimethamine-sulfadiazine was observed. As a result of the regular prescription of pharmacological agents associated with DRESS syndrome, ophthalmologists should be aware of the potentially serious complications of DRESS syndrome.

DRESS syndrome in ophthalmic patients / Síndrome DRESS em pacientes oftalmológicos

ABSTRACT Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal adverse drug reaction associated with skin rash, fever, eosinophilia, and multiple organ injury. A number of pharmacological agents are known to cause DRESS syndrome such as allopurinol, anticonvulsants, vancomycin, trimethoprime-sulfamethoxazole, and pyrimethamine-sulfadiazine. Here, we describe two patients who developed DRESS syndrome during ocular treatment. The first case was being treated for late postoperative endophthalmitis with topical antibiotics, intravenous cephalothin, meropenem, and intravitreal injection of vancomycin and ceftazidime before symptoms developed. We were unable to identify the causal drug owing to the large number of medications concurrently administered. The second case presented with DRESS syndrome symptoms during ocular toxoplasmosis treatment. In this case, a clearer association with pyrimethamine-sulfadiazine was observed. As a result of the regular prescription of pharmacological agents associated with DRESS syndrome, ophthalmologists should be aware of the potentially serious complications of DRESS syndrome.

RESUMO Síndrome DRESS (drug reaction with eosinophilia and systemic symptoms) é uma reação adversa a medicamentos rara e potencialmente fatal, associada à rash cutâneo, febre, eosinofilia e lesão de múltiplos órgãos. Algumas drogas podem desencadeá-la, como: alopurinol, anticonvulsivantes, vancomicina, sulfametoxazol-trimetoprim, sulfadiazina-pirimetamina, entre outras. Descrevemos dois casos que desenvolverem DRESS síndrome durante tratamento ocular. O primeiro caso apresentou os sintomas durante tratamento para endoftalmite pós-operatória tardia com antibióticos tópicos, cefalotina e meropenem intravenosos e injeção intravítrea de vancomicina e ceftazidima; não podemos identificar a droga causadora, pois múltiplas medicações foram utilizadas. O segundo caso desenvolveu os sintomas durante tratamento clássico para toxoplasmose ocular, então a associação com sulfadiazina-pirimetamina foi mais clara. Como muitos oftalmologistas prescrevem regularmente drogas que podem desencadear a síndrome DRESS, esse diagnóstico deve ser lembrado já que pode levar a sérias complicações.

Atypical presentation of pilomatrixoma in the tarsal conjunctiva / Apresentação atípica de pilomatricoma na conjuntiva tarsal

ABSTRACT Pilomatrixoma is a rare benign tumor, which usually affects young women in the head and neck region. The eyelid is a common site of the disease, though it is very rare in the tarsal conjunctiva.The pilomatrixoma has clinical pleomorphism, which confuses this disease with other similar conditions. The diagnosis is made by pathological examination in most cases. The treatment is surgical, performed by total excision of the lesion with clear margins and recurrence is very uncommon.We present an unusual case of probable recurrent pilomatrixoma in the left upper tarsal conjunctiva.

RESUMO O pilomatricoma é uma neoplasia benigna rara, geralmente acomete mulheres jovens, ocorrendo na região da cabeça e pescoço. A pálpebra é um local comum de aparecimento do pilomatricoma, contudo, seu aparecimento na conjuntiva tarsal é muito raro. O diagnóstico é feito pelo exame anátomo-patológico na maioria dos casos, pois seu pleomorfismo clínico o confunde com outras alterações. O tratamento é cirúrgico, realizado por meio da exérese total da lesão com margens livres. São raros os casos de recidiva. Apresentamos um caso atípico de provável recidiva de pilomatricoma, localizado na conjuntiva tarsal superior à esquerda.

Medial rectus muscle myositis as an atypical presentation of mucosa-associated lymphatic tissue lymphoma: a case report.

Here we describe the rare case of a 55-year-old man with medial rectus muscle myositis as an atypical presentation of non-Hodgkin B-cell mucosa-associated lymphoma (MALT). Pathology and immunohistochemistry of the affected muscle confirmed the diagnosis of a neoplasm. The primary etiology of orbital myositis is Graves' ophthalmopathy, but several other diseases may cause this clinical presentation. Therefore, the neoplastic causes must be eliminated from the differential diagnoses. non-Hodgkin B-cell mucosa-associated lymphoma is the most common histological type of lymphoma in the orbit, with the conjunctiva and lacrimal glands being the most commonly affected sites. However, it may also present in atypical forms involving others sites and tissues.

Medial rectus muscle myositis as an atypical presentation of mucosa-associated lymphatic tissue lymphoma: a case report / Miosite do músculo reto medial como forma atípica de apresentação de linfoma tipo MALT: relato de caso

Here we describe the rare case of a 55-year-old man with medial rectus muscle myositis as an atypical presentation of non-Hodgkin B-cell mucosa-associated lymphoma (MALT). Pathology and immunohistochemistry of the affected muscle confirmed the diagnosis of a neoplasm. The primary etiology of orbital myositis is Graves' ophthalmopathy, but several other diseases may cause this clinical presentation. Therefore, the neoplastic causes must be eliminated from the differential diagnoses. non-Hodgkin B-cell mucosa-associated lymphoma is the most common histological type of lymphoma in the orbit, with the conjunctiva and lacrimal glands being the most commonly affected sites. However, it may also present in atypical forms involving others sites and tissues.

Descrevemos um raro caso de miosite do músculo reto medial como forma atípica de apresentação de linfoma não-Hodgkin de células B tipo MALT. A anatomia patológica e imuno-histoquímica do músculo afetado confirmaram o diagnóstico definitivo do caráter neoplásico da doença. As miosites orbitárias têm como principal etiologia a oftalmopatia de Graves, porém diversas outras causas podem apresentar-se dessa forma. Sendo assim, as causas neoplásicas devem ser descartadas. O linfoma não-Hodgkin de células B tipo MALT é o tipo histológico mais comum de linfoma orbitário, as regiões mais frequentemente acometidas são a conjuntiva e glândula lacrimal. No entanto, pode apresentar-se com formas clínicas atípicas, acometendo outras regiões e tecidos.

Comparative study of ophthalmological and serological manifestations and the therapeutic response of patients with isolated scleritis and scleritis associated with systemic diseases / Estudo comparativo entre as manifestações oftalmológicas, sorológicas e resposta terapêutica de pacientes com esclerite isolada e esclerite associada a doenças sistêmicas

INTRODUCTION: Scleritis is a rare, progressive and serious disease, the signs of which are inflammation and edema of episcleral and scleral tissues and is greatly associated with systemic rheumatoid diseases. PURPOSE: To perform a prospective and comparative study between ophthalmologic manifestations, serologic findings and therapeutic response of patients with isolated scleritis and scleritis associated with systemic rheumatoid disease. METHODS: Thirty-two outpatients with non-infectious scleritis were studied, from March 2006 to March 2008. The treatment was corticoid eye drops associated with anti-inflammatory agents, followed by systemic corticoids and immunosuppressive drugs if necessary, was considered successful after six months without scleritis recurrence. RESULTS: Fourteen of 32 patients had scleritis associated with systemic rheumatoid disease, of which nine had rheumatoid arthritis, two systemic lupus erythematosus, one Crohn's disease, one Behçet's disease and one gout. There were no difference in relation to involvement and ocular complications, there was predominance of nodular anterior scleritis and scleral thinning was the most frequent complication. The scleritis associated with systemic rheumatoid disease group had 64.3 percent of autoantibodies, versus 27.8 percent among those with isolated scleritis and this difference was statistically significant. In the isolated scleritis group 16.7 percent used anti-inflammatory, 33.3 percent corticosteroids, 27.8 percent corticosteroids with one immunosuppressive drug, 5.5 percent two immunosuppressive drugs, 16.7 percent corticosteroids with two immunosuppressive drugs and 33.3 percent pulse of immunosuppressive drugs, there was remission in 88.9 percent. In the scleritis associated with systemic rheumatoid disease group 7.1 percent used anti-inflammatory, 7.1 percent corticosteroids, 50 percent corticosteroids with one immunosuppressive drug, 7.1 percent two immunosuppressive drugs and 22.2 percent pulse of immunosuppressive drugs, 100 percent had treatment success. CONCLUSION: Prevalence of unilateral nodular scleritis was noted in both groups and higher rates of all the parameters tested were noted in the scleritis associated with systemic rheumatoid disease group. There were no differences between the groups with respect to the use of immunosuppressive drugs and therapeutic response, which was fully satisfactory in the scleritis associated with systemic rheumatoid disease group and satisfactory in the isolated scleritis group.

INTRODUÇÃO: Esclerite é uma doença grave, rara e progressiva, que envolve inflamação e edema dos tecidos episcleral superficial, profundo e escleral e está associada com doenças sistêmicas reumatológicas em muitos casos. OBJETIVOS: Realizar um estudo prospectivo comparativo entre as manifestações oftalmológicas, achados sorológicos e resposta terapêutica de pacientes com esclerite isolada e com esclerite associada a doenças sistêmicas reumatológicas. MÉTODOS: Trinta e dois pacientes com esclerite não infecciosa participaram do estudo, de março de 2006 a março de 2008. O tratamento realizado baseou-se no uso de colírios de corticoides associados aos anti-inflamatórios não-hormonais, seguidos de corticoides sistêmicos e imunossupressores, se necessário. O sucesso do tratamento foi considerado como seis meses sem crises de esclerite. RESULTADOS: Quatorze dos 32 pacientes apresentaram esclerite associada à doença sistêmica, dos quais nove com artrite reumatóide, dois com lúpus eritematoso sistêmico, um com doença de Crohn, um com doença de Behçet e um com gota. Não houve diferenças em relação ao envolvimento ocular e suas complicações, predominando a esclerite anterior nodular e o afinamento escleral, respectivamente. O grupo com esclerite associada a doenças sistêmicas apresentou 64,3 por cento de positividade de autoanticorpos contra 27,8 por cento no grupo com esclerite isolada, sendo tal diferença estatisticamente significante. No grupo com esclerite isolada, 16,7 por cento fez uso de apenas anti-inflamatórios, 33,3 por cento de corticoide sistêmico, 27,8 por cento de corticoide com um imunossupressor, 5,5 por cento dois imunossupressores, 16,7 por cento corticoide com dois imunossupressores e 33,3 por cento pulsoterapia com imunossupressor; sendo que houve sucesso do tratamento em 88,9 por cento. No grupo com esclerite associada à doença sistêmica, 7,1 por cento fez uso de anti-inflamatórios, 7,1 por cento corticoide sistêmico, 50 por cento corticoide com um imunossupressor, 7,1 por cento dois imunossupressores e 22,2 por cento pulsoterapia com imunossupressor; com 100 por cento de sucesso no tratamento nesse grupo. CONCLUSÃO: Em ambos os grupos houve predomínio da esclerite nodular unilateral e o grupo com esclerite associada a doença sistêmica apresentou taxas maiores de todos os autoanticorpos testados. Não houve diferença entre os grupos em relação ao uso de imunossupressores e à resposta terapêutica, a qual foi totalmente satisfatória no grupo com esclerite associada à doença sistêmica e satisfatória no grupo com esclerite isolada.

Tumor de células granulares no esôfago: relato de 2 casos e revisão de literatura / Granular cells tumor of the esophagus: report of two cases and review of literature

O tumor de células granulares (TCG) ou mioblastoma é um tumor benigno e raro, descrito pela primeira vez por Abrikossoff em 1926 com o relato de cinco casos localizados na língua. Cinco anos mais tarde, o mesmo autor também foi o pioneiro a descrever o TCG de localização esofágica. Desde a sua primeira descrição, o TCG já foi encontrado em diversas regiões do corpo humano, como pele, mama, trato respiratório, sistema biliar, trato gastrointestinal, tireoide, sistema nervoso central e vagina. Sua localização gastrointestinal corresponde a 5-11% dos casos, sendo que um terço destes ocorre no esôfago. A literatura descreve presença de TCG em 0,012 a 0,033% das endoscopias digestivas altas realizadas, sendo que tal proporção se repete em materiais de autópsias. O objetivo do presente trabalho é relatar dois casos de TCG esofágico, discutindo os achados clínicos, endoscópicos e patológicos e realizar uma revisão da literatura sobre o assunto.

Introduction: granular cell tumor (GCT) is a rare benign tumor. About 5-11% of cases are located in gastrointestinal tract, and of these one-third occurs in the esophagus. Objective: to report two cases of esophageal GCT and conduct a review of the literature on the subject. Cases: we present two cases of GCT of the esophagus, both in females (42 and 53 years) with dyspeptic complaints and the diagnosis was confirmed by biopsy during endoscopy. We adopt a conservative approach with endoscopic resection in the first case and clinical in the second, both with good outcome. Discussion: the TCG has no gender preference, being more common in blacks about 45 years. It mainly affects the distal esophagus (65%), there may be multiple lesions in the esophagus and focusing on multiple sites in the body. Half of the patients are asymptomatic, but there may be complaints as dysphagia, retrosternal pain, heartburn, epigastric pain, nausea and vomiting and the presence of symptoms is directly related to the size and multiplicity of lesions. Because its benign behavior, the current trend is expectant management in asymptomatic cases and tumors smaller than 1 cm, with periodic endoscopic follow-up for 1 to 2 years. In symptomatic cases, tumors larger than 1 cm, rapidly growing, or circumferential margins infiltrated resection is recommended, especially endoscopic polypectomy.

Comparative study of ophthalmological and serological manifestations and the therapeutic response of patients with isolated scleritis and scleritis associated with systemic diseases.

INTRODUCTION: Scleritis is a rare, progressive and serious disease, the signs of which are inflammation and edema of episcleral and scleral tissues and is greatly associated with systemic rheumatoid diseases. PURPOSE: To perform a prospective and comparative study between ophthalmologic manifestations, serologic findings and therapeutic response of patients with isolated scleritis and scleritis associated with systemic rheumatoid disease. METHODS: Thirty-two outpatients with non-infectious scleritis were studied, from March 2006 to March 2008. The treatment was corticoid eye drops associated with anti-inflammatory agents, followed by systemic corticoids and immunosuppressive drugs if necessary, was considered successful after six months without scleritis recurrence. RESULTS: Fourteen of 32 patients had scleritis associated with systemic rheumatoid disease, of which nine had rheumatoid arthritis, two systemic lupus erythematosus, one Crohn's disease, one Behçet's disease and one gout. There were no difference in relation to involvement and ocular complications, there was predominance of nodular anterior scleritis and scleral thinning was the most frequent complication. The scleritis associated with systemic rheumatoid disease group had 64.3% of autoantibodies, versus 27.8% among those with isolated scleritis and this difference was statistically significant. In the isolated scleritis group 16.7% used anti-inflammatory, 33.3% corticosteroids, 27.8% corticosteroids with one immunosuppressive drug, 5.5% two immunosuppressive drugs, 16.7% corticosteroids with two immunosuppressive drugs and 33.3% pulse of immunosuppressive drugs, there was remission in 88.9%. In the scleritis associated with systemic rheumatoid disease group 7.1% used anti-inflammatory, 7.1% corticosteroids, 50% corticosteroids with one immunosuppressive drug, 7.1% two immunosuppressive drugs and 22.2% pulse of immunosuppressive drugs, 100% had treatment success. CONCLUSION: Prevalence of unilateral nodular scleritis was noted in both groups and higher rates of all the parameters tested were noted in the scleritis associated with systemic rheumatoid disease group. There were no differences between the groups with respect to the use of immunosuppressive drugs and therapeutic response, which was fully satisfactory in the scleritis associated with systemic rheumatoid disease group and satisfactory in the isolated scleritis group.