Cases reported "Chronic Disease"

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1/74. Systemic lupus erythematosus with optical neuromyelitis (Devic's syndrome). A case with a 35-year follow-up.

    Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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2/74. Hypertrophic discoid lupus erythematosus of the conjunctiva.

    PURPOSE: To report the ophthalmic manifestations of hypertrophic discoid lupus erythematosus of the conjunctiva. METHOD: Case report and review of biopsy results. RESULTS: A 58-year-old woman with a history of chronic blepharoconjunctivitis presented with an unusual raised conjunctival lesion. Previous biopsy slides were reviewed and interpreted as diagnostic of discoid lupus erythematosus, hypertrophic or verrucous type. Both blepharoconjunctivitis and the raised conjunctival lesion resolved with hydroxychloroquine therapy. CONCLUSIONS: A raised conjunctival mass in the context of refractory blepharoconjunctivitis should elicit suspicion for discoid lupus erythematosus. The hypertrophic variant of this disease can affect the conjunctiva.
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ranking = 1.1227994730224
keywords = lupus erythematosus, erythematosus, lupus
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3/74. A case of SLE with acute, subacute and chronic cutaneous lesions successfully treated with dapsone.

    We describe a patient with systemic lupus erythematosus (SLE) who exhibited severe cutaneous involvement characterized by the simultaneous presence of acute, subacute and discoid lesions in association with anti-S1 antibodies. After she failed to respond to chloroquine, medium to low dose steroids, steroid pulses, retinoids and cyclophosphamide, the patient was treated with dapsone and a dramatic improvement in the cutaneous lesions was seen after only one month.
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ranking = 0.17920015056501
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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4/74. Macroscopic and microscopic mucinosis in chronic sclerodermoid graft-versus-host disease.

    Secondary cutaneous mucinosis is a well-recognized feature of connective tissue diseases such as lupus erythematosus and dermatomyositis. We report the first three cases of dermal mucinosis in association with severe chronic cutaneous graft-versus-host disease of the sclerodermoid variety. One patient had clinical changes due to abundant mucin accumulation within the papillary dermis (mucinoma). In the other two patients histological examination revealed extensive deposits of mucin predominantly within the reticular dermis. The microscopic appearances were striking, with numerous vacuolated spaces interspersed between collagen bundles. We speculate that this appearance is the result of ground substance becoming trapped within grossly sclerodermoid connective tissue.
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ranking = 0.16039992471749
keywords = lupus erythematosus, erythematosus, lupus
(Clic here for more details about this article)

5/74. magnetic resonance imaging in the evaluation of patients with aseptic meningoencephalitis and connective tissue disorders.

    OBJECTIVE: To describe the role of magnetic resonance imaging (MRI) in the evaluation of patients with chronic and recurrent aseptic meningitis. METHOD: A retrospective study of five patients with aseptic meningoencefalitis diagnosed by clinical and CSF findings. CT scans showed without no relevant findings. RESULTS: MRI showed small multifocal lesions hyperintense on T2 weighted images and FLAIR, with mild or no gadolinium enhancement, mainly in periventricular and subcortical regions. meningoencephalitis preceded the diagnosis of the underlying disease in four patients (Behcet's disease or systemic lupus erythematosus). After the introduction of adequate treatment for the rheumatic disease, they did not present further symptoms of aseptic meningoencephalitis. CONCLUSION: Aseptic meningoencephalitis can be an early presentation of an autoimmune disease. It is important to emphasize the role of MRI in the diagnosis and follow-up of these patients.
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ranking = 0.17920015056501
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
(Clic here for more details about this article)

6/74. Atypical chronic graft-versus-host disease following interferon therapy for chronic myeloid leukaemia relapsing after allogeneic BMT.

    We report a 54-year-old woman who received interferon alpha for haematological relapse of Ph-positive CML, 7 years after allogeneic BMT from an HLA-identical brother. Eighteen months after relapse, cytogenetic and molecular remission was achieved. She received interferon therapy for 25 months and it was discontinued when she developed skin lesions on her face and trunk, dysphagia and fever with respiratory failure and bilateral patchy airspace consolidation of the lung without microbiologic findings. Histologic features showed discoid lupus erythematosis, oesophagitis with pseudomembranes and a mixed pattern of lymphocytic bronchiolitis involving the alveoli and interstitial spaces all compatible with chronic GVHD. The patient was commenced on immunosuppressive therapy with complete clinical and radiological resolution. The available evidence supports an atypical presentation of chronic GVHD and suggests a role for interferon alpha in the pathogenesis of GVHD. To the best of our knowledge, this is the first case reported of severe chronic GVHD occurring during the course of interferon therapy for relapsed CML.
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ranking = 0.0079983385947587
keywords = lupus
(Clic here for more details about this article)

7/74. hypersensitivity pneumonitis with normal high resolution computed tomography scans.

    A case of symptomatic hypersensitivity pneumonitis with normal high resolution computed tomography (CT) scans is presented. The patient, a 32-year-old man with systemic lupus erythematosus, had a chronic, progressive history of respiratory symptoms, abnormal findings on examination and abnormal pulmonary function tests but normal high resolution CT scans of the chest. diagnosis was made through open lung biopsy. Clinical improvement was seen on removal of the offending antigen. The literature on the utility of high resolution CT scans in hypersensitivity pneumonitis is reviewed.
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ranking = 0.17920015056501
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
(Clic here for more details about this article)

8/74. Intense pulsed light treatment for chronic facial erythema of systemic lupus erythematosus: a case report.

    BACKGROUND: A 33-year-old woman, who had been diagnosed previously with systemic lupus erythematosus, presented with chronic erythema and rosacea of the face. The patient suffered from flushing and burning of the facial skin and sought prior treatment with anti-malarial drugs. METHOD: After various treatment options were discussed with the patient, she agreed to undergo intense pulsed light therapy. RESULTS: Improvement was noted after the first session and 75% clearance was observed at 1 month after a second session. There were no adverse effects associated with the treatment. One year later it was observed that the results of the two treatments had been maintained. CONCLUSION: This case report suggests that there is another safe and efficacious alternative for treatment of chronic erythema and rosacea.
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ranking = 0.87720052697755
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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9/74. Chronic infection due to fusarium oxysporum mimicking lupus vulgaris: case report and review of cutaneous involvement in fusariosis.

    A 67-year-old female presented with a 20-year-old lesion involving the right ear and preauricular area mimicking tuberculous lupus. fusarium oxysporum infection was confirmed by biopsy studies and cultures. The biopsy specimen showed an unusually extensive dermal invasion with fungal hyphae. This is an uncommon clinical presentation for fusarium infection in a healthy patient. When referred to us, the patient had received antifungal therapy with itraconazole without any benefit. Improvement was obtained with fluconazole therapy. The spectrum of cutaneous involvement related to fusarium spp. includes toxic reactions, colonization, superficial indolent infection, deep cutaneous or subcutaneous infections and disseminated infection.
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ranking = 0.039991692973794
keywords = lupus
(Clic here for more details about this article)

10/74. Unilateral chronic thromboembolic pulmonary disease associated with combined inherited thrombophilia.

    Chronic thromboembolic pulmonary hypertension (CTEPH) is considered to be an extreme variant of pulmonary thromboembolism. The underlying mechanisms for the failure of thrombus resolution are still unclear. In looking for inherited thrombophilia, an association with a lupus anticoagulant has been described repeatedly, and single cases of anticoagulant deficiencies (ie, antithrombin [AT], protein c, and protein s) have been reported. We describe a young patient with type I AT deficiency, the heterozygous prothrombin G20210A mutation, and unilateral chronic thromboembolic pulmonary disease presenting after a single thrombotic event. Pulmonary vascular patency was restored successfully by surgical pulmonary thromboendarterectomy. This case is unique because unilateral CTEPH is extremely uncommon, and it illustrates the severe clinical sequelae of the cosegregation of inherited thrombophilic defects.
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ranking = 0.0079983385947587
keywords = lupus
(Clic here for more details about this article)
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