Acute dermatomyositis with subcutaneous generalized edema

The authors report a 40-year-old Caucasian man with relapsing muscle and skin involvement of dermatomyositis treated with high-dose corticosteroids, taken orally, and methotrexate and human gamma globulin, both administered intravenously. After 4 months of aggressive treatment, he presented with generalized edema, considered secondary to dermatomyositis. Aggressive immunosuppression did not stop disease progression. The literature concerning anasarca due to inflammatory myopathies is revised.25689890

Adult-onset still disease in southeast Brazil

Background: Adult-onset Still disease (AOSD) has been described all over the world. Clinical presentations and prognosis have varied in different studies. Objective: The objective of this study was to determine the clinical presentation and the evolution of AOSD at a tertiary referral center in southeast Brazil. Methods: The clinical records of 16 patients were retrospectively studied to determine symptoms at diagnosis, follow up, and the medication prescribed. Results: The mean age at onset was 30.8 years (range, 24-55 years; standard deviation [SD], 9.2 years) with a slight male prevalence (54.2%). All patients presented constitutional symptoms, fever, and skin rash. Liver involvement was observed in all cases, with hepatomegaly in 81.3%, increased liver enzymes in 50.0%, and hypergammaglobulinemia in 68.8%. Cardiac involvement was observed in 12.6%, pleuritis in 6.3%, and renal involvement in 25.0%. All patients presented leukocytosis with a predominance of neutrophils. Elevated ferritin levels were observed in 56.3%, and these levels were normalized after disease remission. Initial treatments included nonsteroidal anti inflammatory drugs and low-dosage corticosteroids in all patients; 43.8% also needed methotrexate. In 25.0% of cases, a monocyclic disease was observed; others had recurrent episodes. After a follow up of 6.9 years (SD, 1.2 years), carpal ankylosis was the main articular sequel, observed in 53.6% of the patients. Conclusion: AOSD is rare in southeast Brazil. Although less severe systemic manifestations, like serositis and pneumonitis, were observed, reversible liver involvement was common; the frequency of recurrent disease and carpal ankylosis was higher than in previous studies.112768

Protein-losing enteropathy associated with systemic lupus erythematosus: response to cyclophosphamide

Protein-losing enteropathy is a rare manifestation of systemic lupus erythematosus (SLE) leading to hypoalbuminemia and anasarca. We report the case of a woman with SLE who presented chronic hypoalbuminemia diagnosed as protein-losing enteropathy associated with SLE. She was refractory to prednisone and azathioprine administration but showed good response to cyclophosphamide. The diagnosis and management of hypoalbuminemia in lupus-associated enteropathy are discussed.25213513

Camurati-Engelmann disease: failure of response to bisphosphonates: report of two cases

Camurati-Engelmann disease is a rare bone disorder characterized by cortical thickening of the diaphysis of tubular bones, with sparing of the epiphysis. It has variable degrees of penetrance and expression, but may be very disabling for the affected individuals who manifest the painful symptoms. The authors report on two women with typical presentation of severe Camurati-Engelmann disease whose treatment with bisphosphonates failed to add any improvement beyond that elicited by corticosteroids alone.24439840

Differential diagnosis of optic neuritis in a patient with ankylosing spondylitis

The authors report a 38-year-old white man with ankylosing spondylitis (AS) who presented with recurrent ischaemic optic neuritis. The initial diagnosis was of multiple sclerosis, but further investigation showed serology and cerebrospinal fluid culture positive for syphilis. After treatment for tertiary syphilis with penicillin, there was complete remission of the ocular symptoms. This case illustrates the differential diagnosis of optic neuritis in AS and supports the relevance of investigating infectious diseases in the management of ischaemic optic neuritis.24424724

Desnutrição neonatal e microbiota normal da cavidade oral em ratos Neonatal malnutrition and normal microbiota of the oral cavity in rats

OBJETIVO: Avaliar a influência da desnutrição neonatal sobre o padrão e o crescimento de bactérias aeróbias, da microbiota normal da cavidade oral, em ratos Wistar adultos. MÉTODOS: O material da cavidade oral foi coletado através de swabs embebidos em 40µL de solução salina estéril e colocados em tubos estéreis contendo 960µL de brain heart infusion. Posteriormente, fez-se homogeneização de cada uma amostra. Então, destes 1.000µL, retirou-se 1µL e este foi semeado em placas de Petri contendo Agar-sangue e Levine para isolamento e identificação de bactérias Gram+ e Gram-, respectivamente. Essas placas foram incubadas em estufa bacteriológica a 37ºC, 48 horas, e as unidades formadoras de colônias que cresceram foram contadas e seus percentuais calculados. Para a bacterioscopia foram confeccionadas lâminas coradas pelo método de Gram. RESULTADOS: Do 5º ao 21º dia de vida os pesos corporais do grupo desnutrido (33,6g:42,8g, desvio-padrão=27,2g) foram menores (p<0,001) que os do grupo nutrido (52,5g:56,3g, desvio-padrão=24,0g). Dos 60 aos 120 dias de vida os pesos foram reduzidos no grupo desnutrido (248,2g:290,2g, desvio-padrão=220,2g), quando comparados aos do grupo nutrido (306,2g:319,4g, desvio-padrão=219,5g) teste Mann Whitney, p<0,05. A microbiota foi composta de menor número de bactérias no grupo nutrido (4,2x10(6) unidades formadoras de colônias, desvio-padrão=1,2x10(6) unidades formadoras de colônias de bactérias/mL), comparado ao grupo desnutrido (7,4x10(6) unidades formadoras de colônias, desvio-padrão=1,0x10(6) unidades formadoras de colônias de bactérias/mL) teste "t", (p=0,026). Das bactérias isoladas em culturas, observou-se um padrão bacteriano semelhante nos dois grupos, com 70% de bactérias gram-positivas e 30% de bactérias gram-negativas. CONCLUSÃO: A desnutrição neonatal foi efetiva em reduzir o peso corporal e aumentar o crescimento bacteriano. Embora o padrão bacteriano não tenha sido alterado, o aumento do crescimento bacteriano pode induzir a um desequilíbrio na microbiota oral desfavorecendo assim, o hospedeiro.<br>OBJECTIVE: To evaluate the influence of neonatal malnutrition on the pattern and growth of aerobic bacteria of the normal bacterial flora of the oral cavity in adults Wistar rats. METHODS: In the present study, the material of the oral cavity was collected through swabs soaked in 40µL of sterile saline solution. After the collection, each swab was placed in a sterile tube containing 960µL of brain heart infusion. Later, the samples were homogenized. Then, from the 1.000µL, 1µL was collected with a gauged loop to be sowed in Petri dishes containing Agar-blood and Agar-Levine, for the isolation and identification of the Gram-positive and Gram-negative bacteria respectively. The plates were placed into a bacteriological incubator, 37ºC, for 48 hours and the colony-forming units that grew were counted and their percentages were calculated. For bacterioscopy, slides were stained with the Gram method. RESULTS: From the 5th to the 21st day of life, body weight of the undernourished group (33.6g:42.8g, standard deviation=27.2g) was smaller (p<0.001) than that of the nourished group (52.5g:56.3g, standard deviation=24.0g). From the 60th to the 120th days of life the body weight of the undernourished group (7.4.x.10(6) colony-forming units, standard deviation=1,0x10(6) colony-forming units bacteria/mL) decreased when compared to that of the nourished group (306.2g:319.4g, standard deviation=219.5g), Mann-Whitney test, p<0,05. The microbiota was composed of a smaller number of bacteria in the nourished group (4.2 x10(6) colony-forming units, standard deviation=1.2x10(6) colony-forming units bacteria/mL) when compared with the undernourished group (7.4x10(6) colony-forming units, standard deviation=1.0x10(6) colony-forming units bacteria/mL), Student's "t" test (p=0.026). From the bacteria isolated in cultures, a similar bacterial pattern was observed in both groups, with 70% of Gram-positive bacteria and 30% of Gram-negative bacteria. CONCLUSION: Neonatal malnutrition was effective in decreasing body weight and increasing bacterial growth. Although the bacterial pattern was not altered, the increase in bacterial growth may induce an imbalance of the oral microbiota, being a threat to the host