[Cutaneous vasculitis detected by shunt nephritis].
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Biomedical subjects
Publications and source records attributed to P Disdier.
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We report a case of mitochondrial myopathy discovered in a 55-year old woman who was being investigated for the cause of her asthenia. Physical examination showed ptosis of the upper eyelid and proximal muscle deficit. Histological examination of a muscle biopsy disclosed rare fibres with mitochondrial aggregates. Biochemical exploration of muscle tissue revealed a double enzyme deficit involving complexes I and IV of the respiratory chain. Clinical improvement was obtained after the patient was put on coenzyme Q10. We conclude that a diagnosis of mitochondrial myopathy must be considered in patients, including middle-aged adults, presenting with muscular asthenia.
To our knowledge, the occurrence of extensive pharyngeal necrosis in patients with dermatomyositis has not yet been reported. We report such a case characterized by a vast ulceration of the posterior laryngeal wall, extending from the velum palati to the larynx and responsible for both dysphagia and dysphonia. Treatment with an immunosuppressant had no effect on the lesions.
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We report a case of neurosarcoidosis characterized by central nervous system involvement 6 years after the onset of the disease. Our case is remarkable for the gradual development of an amnesic syndrome. Although neurological examinations gave normal results, the semeiological profile of the cognitive disorder suggests the presence of a hypothalamic sarcoid lesion involving the basal forebrain.
In patients treated for breast carcinoma, unilateral lymphedema of the upper limb is usual. However, to the authors' knowledge, lower limb lymphedema has never been reported as a complication of breast carcinoma therapy. They report here the first case of a radiation-induced constrictive pericarditis revealed by severe lower limbs lymphedema. A 60-year-old woman was treated for left breast carcinoma with quadrantectomy, axillary lymphadenectomy, and combined radio chemotherapy (60 grays). Three and a half years later she suffered from a diffuse and increasing lower limbs lymphedema, which became huge and disabling. Radiation-induced constrictive pericarditis was evidenced by right cardiac cavities catheterization. A dramatic improvement was rapidly obtained after pericardectomy. Histopathologic analysis of the pericardium did not reveal neoplastic cells. Radiation-induced constrictive pericarditis is usually responsible for lower limbs edema, but lymphedema is exceptional. This case highlights the need to search for a constrictive pericarditis also in the case of lower limbs lymphedema, particularly in a patient treated with mediastinal radiotherapy or combined radio chemotherapy.
BACKGROUND: Behçet's disease is an uncommon cause of prolonged fever. This misleading presentation is illustrated by a new case where fever occurred after polyvalent vaccination. CASE REPORT: A 39-year-old female patient was seen for prolonged fever after returning from a trip to Africa. She had received polyvalent vaccinations prior to leaving France. At admission fever had persisted for 2 months. The diagnosis of Behçet's disease was finally established on the basis of clinical signs, buccogenital aphtosis and pathergy. Colchicine was partially effective against although low-grade persisted in the evening. DISCUSSION: Clinicians should be aware that Behçet's disease can provoke long-term fever which can sometimes be an inaugural sign. Sensitivity tests may be useful to identify the trigger, for example polyvalent vaccination.
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Infections and/or immune-mediated reactions may occur after intravesical instillation of bacillus Calmette-Guérin for the treatment of bladder carcinoma. We report herein a cryoglobulinemia vasculitis occurring after intravesical BCG instillation for a superficial papillary transitional cell bladder carcinoma. The patient, an 80-year-old man, presented peripheral ischemia 10 days after the second course of intravesical BCG instillation. Biological evaluation revealed autoimmune thrombocytopenia, hypergammaglobulinemia, low C3 and C4 complement fraction levels related to mixed cryoglobulinemia and lupus anticoagulant. The patient was treated with heparin and prostacyclins with a good outcome. All of the immune anomalies spontaneously regressed within 3 months. To our knowledge, cryoglobulinemia has only been reported once in the literature and lupus anti-coagulant has never been reported as a complication of intravesical BCG instillation.