1. Residente do 2º ano de Reumatologia, Brasília, DF 2. Médica estagiária em Reumatologia, Brasília, DF 3. Médico reumatologista, Brasília, DF 4. Médicos reumatologista assistente do Hospital Universitário de Brasília, Brasília,DF 5. Residente do 1º ano de Reumatologia, Brasília,DF introdução A vasculite reumatoide (VR) é uma condição rara e grave. Os achados cutâneos podem ser bastante variados. Descrevemos um raro caso de vasculite reumatoide com evolução cutânea grave. relato de casoMulher, 49 anos, com diagnóstico de AR há 15 anos, em tratamento com metotrexato 15 mg/semana e prednisona 10 mg/dia. Procurou o HUB, relatando, há oito dias, edema e manchas violáceas em perna direita, confluentes, intensamente dolorosas, com vesículas e bolhas de conteúdo seroso. O quadro sugeria erisipela bolhosa, sendo iniciada antibioticoterapia. Em três dias as lesões progrediram para grandes bolhas e úlceras com exsudação e material necrótico. Exames laboratoriais: hemoglobina de 7,7 g/dl, leucometria de 16.400, VHS:60 mm/h e PCR: 35 mg/dl. Biópsia da lesão: intenso infiltrado neutrofílico na derme profunda e tecido subcutâneo, sinais de vasculite neutrofílica em pequenos vasos (necrose fibrinóide da parede, trombose e hemorragia). A cultura de fragmento cutâneo, bem como a hemocultura mostraram Acinetobacter baumanii. Diante do diagnóstico de VR cutânea, com quadro infeccioso subjacente, iniciou-se prednisona 1 mg/kg/dia, ciclofosfamida, via oral, e pentoxifilina. O esquema antimicrobiano foi alterado para imipenem e vancomicina. Houve rápida piora do quadro cutâneo, com necrose extensa dermo-epidérmica e ampla exposição de fáscia e planos musculares profundos, em toda a extensão de membro inferior direito, abaixo do joelho. Optou-se por pulsos de metilprednisolona 500mg/dia por três dias, indicado desbridamento cirúrgico amplo das lesões, além de oxigenioterapia hiperbárica. Após um mês da terapia, houve surgimento de tecido de granulação, o que possibilitou o planejamento de terapia cirúrgica (enxertia) para recobrir a extensa área de necrose.
BACKGROUNDImmunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect several organs with different clinical manifestations and laboratory alterations, which makes its diagnosis challenging. CASE REPORTFemale, 57 years old, diabetic, dyslipidemic and former smoker. In 2019, she had an unintentional weight loss of 24 kg in 4 months with nausea and hyporexia. In the investigation, the computed tomography scan of the abdomen that showed a retroperitoneal mass with soft tissue density measuring 14.4 × 4.9 × 3.2 cm involving the infrarenal abdominal aorta. Mild left hydronephrosis up to the middle third. Lesion biopsy with histopathological report (April/20): IgG pos (large amount of plasma cells); IgG4 pos (in moderate amount of plasma cells containing more than 30 positive cells and IgG4:IG ratio > 40%); with the following findings: fibrosis in a striated pattern, with obliterating phlebitis and dense lymphoplasmacytic infiltrate. After a year of tomographic control, the patient presented an important volumetric reduction of the described retroperitoneal lesion, but with the appearance of ureteropyelocaliceal dilatation on the right and accentuation of pyelocaliceal dilatation on the left. Renal scintigraphy already showed functional exclusion of the left kidney. Due to the bilateral ureteral stenosis, double J was placed bilaterally, with replacement every 3 months, with definitive removal of the double J on the left in June/21 and the right in April/22. The patient is on corticosteroid therapy. She made use of methotrexate, with suspension due to gastric intolerance and azathioprine 100 mg was started with a subsequent increase to 150 mg. Due to left renal exclusion and worsening of right hydronephrosis, it was decided to switch to rituximab (severe manifestation of IgG4-RD). CONCLUSIONRetroperitoneal fibrosis (RPF) is characterized by fibroinflammatory tissue in the retroperitoneum, often anterolateral to the aorta and/or its branches, which may result in hydronephrosis. RPF has been shown to be related to IgG4-RD, with the following criteria: 1) diffuse/localized mass in a single or multiple organs; 2) serum IgG4 levels >135 mg/dL; 3) histological findings of: i) marked infiltration of lymphocytes and plasma cells, and fibrosis; ii) cell infiltration. IgG4 (+) plasma cells: total IgG4/IgG ratio > 40%, >10 cell. IgG4 (+)/field. The first line of treatment in RPF is draining renal decompression associated with high-dose steroid therapy, but exacerbations are common even after treatment is started. Azathioprine, mycophenolate mofetil, and methotrexate are used as steroid-sparing agents. For patients with recurrent or refractory disease, immunobiologicals can be used, such as rituximab.
BACKGROUNDReactive seronegative sacroiliitis secondary to use of isotretinoin is a rare adverse effect. About 16% of patients can evolve with myalgia, arthralgia and low back pain. CASE REPORTTGAP, 15 years old, male, was interned at neurology ward of a tertiary hospital in the Federal District for low back pain. The Rheumatology team was requested, noting inflammatory low back pain about 3 weeks ago, associated with right anterosuperior spinal enthesitis, fevers episodes and elevation of evidence with erythrocyte sedimentation rate (ESR) 44 and C-reactive protein (CRP) of 8.4, in addition to the presence of acne fulminans. He referred that about 11 days ago, had positive NS1 for dengue and history of use for 2 months of isotretinoin, being suspended in the same month of hospitalization. Due to low back inflammatory pain, that have started before the diagnosis of dengue, the arthropathy inflammatory per isotretinoin became the main diagnosis. This medication was discontinued, no steroidal anti-inflammatory started and requested resonance (MRI) of sacroiliac and hip. In return after 1 month in outpatient, there were had improvement frame substance of pain, in addition to CRP 1.7 and improvement in the cutaneous manifestations. In coxofemoral MRI results, and still without result of sacroiliac, it was observed apophysitis in bigger trochanter and anterosuperior iliac spine and light hyposignal area in T1 in sacroiliac. Therefore, the use of anti-inflammatory on demand and observation of the evolution of the clinical condition were chosen. CONCLUSIONDespite the absence of MRI results from sacroiliac, the character of inflammatory low back pain, the presence of fever episodes, enthesitis and increased inflammatory evidence, with improvement after suspension of isotretinoin and prescription of anti-inflammatory and the finding of sacroiliac enhancement in T1, reinforce the rare diagnosis of arthropathy secondary to use of isotretinoin.
BACKGROUNDBehçet's disease (BD) is an inflammatory multisystem disease. Neuro-Behçet's disease (NBD) has variable prevalence depending on the series but can represent around 5-10% of the affected patients in a large series. The diagnostic criteria for NBD are the current diagnosis of BD plus the presence of neurological symptoms not otherwise explained by known systemic or neurological diseases. In this report, we would like to present a case of NBD with an unusual initial presentation of papilledema and intracranial hypertension and emphasize on NBD as rare but important cause of bilateral papilledema due to intracranial hypertension.
BACKGROUNDPatients with SLE appear to have an increased risk for infections due to defects of the immune system. Disease activity is a risk factor for infections, especially when it includes organ involvement such as the kidney. Fungal diseases are common and potentially serious in immunosuppressed patients. Fusarium spp. is an opportunistic fungus. Fusarium infection can be localized, focally invasive, or disseminated and represents a complication of high morbidity and mortality in immunocompromised. We report a case of fusariosis in an immunocompromised patient with refractory lupus nephritis.
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