[The association of lichen planus and primary Gougerot-Sjögren syndrome].
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Biomedical subjects
Publications and source records attributed to B Lorcerie.
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Among cardiac manifestations of Lyme disease, pericardic involvement, as the main symptom, seems to be exceptional. We report two cases of pericarditis with typical clinical features. In both cases, pericarditis was the only clinical sign of the disease. Diagnosis was based on significant high serological antibodies levels with no evidence of any other etiological factor. Penicillin was administered and lead in one case to negativation of the serology. As cardiac involvement can occur without any preexisting cutaneous lesion, the frequency of pericardic involvement in Lyme disease is certainly underestimated.
Two brothers, aged 29 and 31, developed severe type E botulism 12 hours after eating salted herrings. The younger died rapidly, the other survived after treatment with type E botulinus antitoxin under intensive care. Limited electromyographic exploration of the surviving patient showed characteristic signs of presynaptic block. This method may be helpful in the diagnosis of atypical forms of the disease. Type E botulism is uncommon in France. It is almost exclusively transmitted by raw, smoked or salted fish and, for some obscure reason, rarely affects all those who shared the same meal. Contrary to types A and B botulism, it usually responds to its specific antitoxin, which must be administered as early as possible. The other therapeutic measures (tracheostomy, controlled ventilation, tube feeding) are the same for all types.
We report a case of peliosis hepatis in a 47-year old male patient with dermatomyositis treated with azathioprine and corticosteroids. Three months after the combined treatment was initiated, the patient developed right thoracic herpes zoster, agranulocytosis and liver enlargement with signs of portal hypertension. Needle biopsy of the liver revealed peliosis. Azathioprine was withdrawn. The clinical and laboratory abnormalities disappeared progressively. The main causes of peliosis hepatis are considered. Up to now, azathioprine had been held responsible for peliosis hepatis only in renal transplant recipients.
A case of pseudohyperphosphataemia detected in a male patient with multiple IgG myeloma is reported. Phosphataemia, measured by a technique without previous deproteinization, reached 5.66 mM before any treatment and varied with treatment-induced changes in monoclonal immunoglobulin levels. Conversely, normal phosphataemia levels were found in blood samples taken before and after treatment when serum was deproteinized. This pseudohyperphosphataemia resulted from an increase in optic density due to interference between monoclonal immunoglobulin and the molybdic reagent used to determine phosphataemia. A retrospective investigation yielded three similar cases: two in patients with myeloma and one in a patient with non-myelomatous monoclonal dysglobulinaemia. A brief prospective study showed that this phenomenon was relatively frequent, as it was found in 2 out of 9 patients with monoclonal immunoglobulin (IgG in all 9 cases). These data indicate that the finding of marked hyperphosphataemia in subjects with monoclonal dysglobulinaemia should always prompt a control assay performed on deproteinized blood.
Extramedullary plasmocytomas are ubiquitous plasmocytic tumours which are principally located in bones and mucosae but rarely found on the skin. Clinically, they present as purplish-blue cutaneous nodules or, less frequently, as papular or urticarial eruptions. The diagnosis rests on histology and immunostaining. Cutaneous plasmocytomas are usually divided into two types: (1) primary plamocytomas which occur in the absence of myeloma, present as solitary or multiple skin tumours, accompanied or not by monoclonal gammopathy, and have an imperfectly known course and prognosis; (2) secondary plasmocytomas--a case of which is reported here--which appear in the course of a large tumoral mass myeloma and have a very poor prognosis.
A clinical history typical of multisclerosis began in a 20-year old man with transient, then permanent manifestations involving the optic tract and the pyramidal, extrapyramidal and cerebellar systems. The patient died at the age of 62, at the end-stage of a complex clinical situation which included paraplegia, bilateral cerebellar syndrome, optic nerve atrophy, epileptic seizures and dementia. When the patient was 54 years old, laboratory findings suggestive of systemic lupus erythematosus (SLE) were discovered, namely: antinuclear, native anti-DNA, anti-Sm antibodies, circulating anticoagulant, cryoglobulinaemia and low complement level. These abnormalities persisted up to the patient's death, 8 years later, without any non-neurological sign of SLE. Post-mortem examination showed lesions of focal demyelination characteristic of multiple sclerosis, but no evidence of cerebral or extracerebral SLE. This case raises the problem of borderlines or associations between systemic lupus erythematosus and multiple sclerosis. In our case, as in other cases of "lupoid sclerosis" reported in the literature, there was a frank and isolated elevation of serum IgM levels.
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OBJECTIVES: To evaluate the clinical presentation and biological features of primary hyperparathyroidism (pHPT) in very old subjects. METHODS: Thirty patients aged 75 years or more, with pHPT were analyzed retrospectively. The diagnosis of pHPT was based on usual biological signs and was always confirmed by the finding of morphological abnormalities with parathyroid imaging or neck surgical exploration. RESULTS: At initial examination, recent general symptoms and neuromuscular symptoms were observed in 87% and 83% of the cases, respectively. Neuropsychiatric manifestations were noted in 73% of the cases. Confusion and depression were the most frequent findings, observed in 30% and 27% of the cases, respectively. In one of two patients with dementia like state, the mental status improved dramatically after surgery of the parathyroid adenoma. Serum calcium levels were in the normal range in 13% of the cases and equal or below 110 mg/L in 47% of the cases. There was no correlation between serum calcium level and the severity of mental changes. Serum parathyroid hormone determinations were abnormal in 93% of the patients. Thirteen patients underwent surgery with biological improvement in all cases. An ultrasonically guided percutaneous ethanol injection of parathyroid adenoma was performed in 10 patients with a biological cure rate of 50%. CONCLUSION: This study shows the major prevalence and potential severity of neuropsychiatric disorders in pHPT in very old patients. Because neuropsychiatric disturbances can respond favourably to surgical treatment, the clinician must systematically look for pHPT in elderly patients with cognitive impairment.