[Pachydermodactyly: report of a case]. / La pachydermodactylie: une observation.

This 20 year old white patient complained of progressive thickening of his fingers over a period of four years. The second, third and fifth fingers of each hand indeed appeared podgy. The soft tissue on each side of the first phalanx was swollen, firm without adherence to the bone. The skin of the interdigital space was hyperpigmented and lichenified. This case illustrates the clinical and histologic features of digital fibromatosis which are discussed.

[Hypothyroid myopathy: apropos of a case with a great increase of creatine phosphokinase, myoglobinemia and transient kidney failure]. / Myopathie hypothyroïdienne: à propos d'un cas avec élévation majeure de la créatine phosphokinase, myoglobinémie et insuffisance rénale transitoire.

Clinical, usually moderate myopathy, frequently develops in patients with hypothyroidism and may reveal the thyroid dysfunction. Biological signs of myolysis, essentially a, usually moderate, rise in creatine phosphokinase plasma levels, are often found. On the other hand, rhabdomyolysis seems to be exceptional in this context. We report a case where an extremely severe clinical and biological myopathy associated with transient renal failure revealed hypothyroidism due to prolonged intake of amiodarone.

[A new cause of unexplained prolonged fever: estroprogestogen treatments, apropos of 12 cases]. / Une nouvelle cause de "fièvre" prolongée inexpliquée: les traitements oestroprogestatifs. (A propos de 12 cas).

Twelve female patients treated by progestogens with or without estrogens presented with prolonged (1 to 48 months) elevation of body temperature above 37 degrees C but below 38 degrees C. They had usually continued taking their temperature after and initial transitory febrile episode. Most had had multiple diagnostic tests which remained negative. Temperature became normal after cessation of hormonal treatment.

[Systemic reaction induced my minocycline treatment: a report of four patients and a review of the literature]. / Réactions systémiques secondaires à la prise de minocycline. A propos de quatre observations et revue de la littérature.

We report four cases of the side effects of minocycline seen during the last two years in our department. There was one case of drug-related lupus and three cases of hypersensitivity reactions, including one eosinophilic pneumopathy with pericarditis, one nephropathy and one severe, pseudo-infectious episode of high fever, rash, lympadenopathy, hepatitis and eosinophilia. Minocycline is a tetracycline agent widely used for acne therapy in France and all over the world. During the last few years, there has been an increasing number of reports concerning systemic adverse reactions to minocycline, with on the one hand auto-immune disorders (lupus, autoimmune hepatitis, vascularitis with ANCA), occurring after a prolonged course of therapy and reported recently in the last few years, and on the other hand, hypersensitivity reactions (eosinophilic pneumopathies, hepatitis, nephropathies, myocarditis, serum sickness or pseudo-infectious reactions), occurring precociously in the course of therapy, and potentially severe. Although these side effects are uncommon in the context of the high number of patients who have been prescribed the drug, the first-line antibiotic therapy in acne must probably be reconsidered.

[Idiopathic retroperitoneal fibrosis disclosed by nephrogenic diabetes insipidus]. / Fibrose rétropéritonéale idiopathique révélée par un diabète insipide néphrogénique.

Nephrogenic diabetes insipidus is an exceptional complication of incomplete ureteric obstruction with hydronephrosis. We report one case of this condition revealing idiopathic retroperitoneal fibrosis. Pathogenic mechanisms are discussed.

[Hyperthyroidism and hypersecretion of chorionic gonadotropin in gastric adenocarcinoma]. / Hyperthyroïdie et hypersécrétion de gonadotrophine chorionique au cours d'un adénocarcinome gastrique.

We report a case of hyperthyroidism complicating and revealing a metastatized adenocarcinoma of the stomach with high circulating levels of human chorionic gonadotropin and its beta component. To our knowledge, this is the first reported case of a non-trophoblastic tumor with hyperthyroidism and secreting quantities of these hormones. High circulating levels of beta component in such patients have been considered to be a sign of malignancy. The mechanisms of thyroid stimulation could be related to the biological activity of certain forms of the human chorionic gonadotropin.

[Crowned dens syndrome: three new cases]. / Syndrome de la dent couronnée : à propos de trois nouveaux cas.

INTRODUCTION: Crowned dens syndrome is due to a microcrystalline infringement (hydroxyapatite or calcium pyrophosphate) of the retro-odontoidal ligament of atlas, often leading to the erroneous diagnosis of meningitis or spondylitis. We report on three new cases diagnosed from 1996 to 1999. EXEGESIS: The patients complained of cervicalgies, headaches or fever. The initially evoked diagnoses were meningitis, spondylodiscitis or endocarditis. Clinical exam found meningism and an inflammatory syndrome in all patients. Analysis of the cerebro-spinal fluid realised in two cases was normal. The diagnosis of crowned dens syndrome was assessed in two cases by cervical CT scan of C1/C2. In the third case, chondrocalcinosis of a wrist allowed this diagnosis. We report a probably non fortuitous case of crowned dens syndrome associated with genetic hemochromatosis. A non steroidal anti-inflammatory treatment allowed a dramatic regression of clinical symptoms. CONCLUSION: This entity should be better known; it can mimick numerous diagnosis and be responsible for fever in the long course.

[Immunotolerance of RU 41740]. / Immunotolérance du RU 41740.

RU 41740 is an immunomodulator of organic origin to be used preventively against recurrent respiratory infections in both adults and children. The immunological safety of the drug was first studied on NZB-NZW mice which spontaneously develop a disease resembling lupus erythematosus. The survival rate of these mice and the amounts of protein excreted in the urine were not modified by the compound. Clinically, RU 41740 was well tolerated by the adult mice and their offspring. Studies on immunological safety were also conducted in human adults and children. Compared with pre-treatment values, there were no pathological changes in blood count and differential, erythrocyte sedimentation rate, immunoglobulins E, A, M and G levels, apolipoproteins, haptoglobin, haemolytic complement, antinuclear factors, rheumatic serology and anti-red cell antibodies. In addition, the drug was well tolerated by atopic subjects.

["Primary" pulmonary arterial hypertension associated with HIV infection. Two cases]. / Hypertension artérielle pulmonaire "primitive" associée à l'infection par le VIH. Deux observations.

We report two cases of pulmonary arterial hypertension (PAHT) in HIV infected patients who never were, or had ceased to be, drug addicts. A study of these cases and a review of the literature show that this association is not fortuitous and persists after the classical causes of PAHT (pulmonary embolism, toxic factors, cirrhosis) have been excluded. The clinical features and the results of complementary cardiovascular examinations are identical with those of the so-called "primary" PAHT. The prognosis is severe: 50 percent of the patients died of the consequences of PAHT 1 year after the first clinical signs. Histology displays signs of plexogenic pulmonary arteriopathy, as in primary PAHT. In HIV patients pulmonary arterial hypertension occurs independently of the degree of immunodeficiency. Its relation with other HIV-related vasculites and their physiopathology are discussed.

[A new cause of prolonged fever of unknown origin: progesterone substitution therapy]. / Une nouvelle cause de fièvre prolongée inexpliquée: les traitements progestatifs.

OBJECTIVES: Over the last 3 years, we examined 15 patients on progesterone substitution therapy who presented prolonged fever. Many explanations had been put forward before the diagnosis was suggested. We recall the thermogenic effect of progesterone. METHODS: Based on a retrospective analysis, we reported the clinical and biological features of 15 patients with prolonged fever due to progesterone substitution therapy and prospectively followed the temperature curves of 9 menopaused women who received substitution therapy for the first time. RESULTS: The 15 case reports were quite typical. Often onset occurred with an acute infectious episode. Following this episode, the patients continued to take their temperature and discovered persistent fever. The context was often one of anxiety-depression rich in functional symptomatology. Laboratory findings included a normal sedimentation rate in all patients. Several days after treatment withdrawal temperature returned to normal. The prospective study confirmed the thermogenetic effect of almost all progesterone substitution drugs. CONCLUSION: The thermogenic effect of natural progesterone is well known but it must be recalled that all progesterone agents with 5-beta metabolites have the same effect. Since metabolic clearance is long, the thermogenic effect may persist for several days after withdrawal.

[Deep phlebitis of the lower limbs. Diagnostic value of D-dimer assays]. / Phlébites profondes des membres inférieurs. Intérêt diagnostique du dosage des D-dimères.

D-dimers are specific fibrin degradation products which have been measured in plasma and used as a diagnostic test for deep vein thrombosis of the lower limbs. However, the results published so far have often been conflicting. We compared the results of D-dimer assays by the latex method (positivity threshold: 0.5 microgram/ml) in 3 groups of patients: 22 patients with clinically suspected thrombophlebitis confirmed by doppler ultrasound; 22 patients with clinically suspected thrombophlebitis not confirmed by doppler ultrasound; and 17 hospital patients with other diseases serving as controls. The sensitivity of a D-dimer concentration higher than 0.5 microgram/ml was 96 percent and its specificity 69 percent. In an internal medicine unit where the prevalence of thrombophlebitis was 50 percent, the positive predictive value of a D-dimer concentration higher than 0.5 microgram/ml was 81 percent, and its negative predictive value was 94 percent. Thus, because of its low specificity as compared with imaging methods the D-dimer assay test cannot be used to diagnose deep vein thrombosis, but as it has good sensitivity and is rapidly performed, safe and inexpensive it can be used as an emergency examination which, when negative, makes this diagnosis unlikely.

[Antiphospholipid syndrome. 20 cases]. / Syndrome des antiphospholipides. 20 observations.

OBJECTIVES: We analyzed the clinical and biological characteristics as well as the clinical course and outcome observed in 20 patients with antiphospholipid antibodies and clinical signs including thrombosis or repeated spontaneous abortion to better identify the recently described antiphospholipid syndrome. METHODS: We retrospectively studied all patients observed in our unit from 1981 to 1992 who fulfilled the following inclusion criteria: a) at least one episode of arterial or venous thrombosis and/or repeated spontaneous abortions, b) positive for antiphospholipid antibodies. RESULTS: Twenty patients were included, 3 with systemic lupus erythematosus (according to the American Rheumatism Association criteria). Arterial or venous thrombosis occurred in 9 and 16 respectively, including exceptional cases of cerebral phlebitis and thrombosis of dermal capillaries. High blood pressure was recorded in 8. Only 1 or 2 types of antiphospholipid antibodies were found in most patients. Anticardiolipin, a circulating anticoagulant and a false-positive Bordet-Wassermann reaction were found together in only 3 out of 16. In addition, the antibody level varied independently from the thrombotic events. There was no case with a clinical course from primary antiphospholipid syndrome to systemic erythromatosus lupus. The effect to treatment on occurrence of new thrombotic events was studied. Three patients suffered one or more haemorrhagic events during antivitamin K treatment. CONCLUSION: It is difficult to establish a differentiation between primary antiphospholipid syndrome, systemic lupus erythematosus and lupus-like syndromes, and precise methods of identifying antiphospholipid antibodies should be further developed.

1,25-dihydroxyvitamin D3 potentiates the in vitro inhibitory effects of cyclosporin A on T cells from rheumatoid arthritis patients.

Treatment of rheumatoid arthritis (RA) with cyclosporin A (CsA) has been successful, but the adverse effects of the drug have limited its use. We investigated the capacity of another immunosuppressive agent, 1,25-dihydroxyvitamin D3 [1,25(OH)2D3], to augment the effects of CsA on in vitro T cell functions. Exposure of CD4+ cells from RA patients or from normal subjects to either molecule alone resulted in a dose-dependent inhibition of phytohemagglutinin stimulation and interleukin-2 (IL-2) production that was more pronounced in cells from RA patients than in cells from normal subjects. Moreover, the action of CsA and 1,25(OH)2D3 on RA patient T cell functions in vitro was synergistic. Thus, in the presence of the vitamin D3 metabolite, only one-hundredth the concentration of CsA was required to produce the same effect on IL-2 production as that produced by CsA alone. IL-2 receptor expression was also reduced by the addition of both drugs. In contrast, IL-1 production by RA monocytes was not affected by CsA and 1,25(OH)2D3, either together or alone, and addition of IL-1 did not restore the ability of CD4+ cells from RA patients to secrete IL-2. Exogenous IL-2 reversed a large part of the inhibitory effect induced by both CsA and 1,25(OH)2D3, indicating that the immunosuppressive properties of these agents are mediated by the inhibition of IL-2 secretion. This synergy between 2 molecules that are biochemically very different suggests the existence of one or several sites of interaction that take place during the early stages of T cell activation.

[Primary Nocardia asteroides deltoid abscess in treated Horton disease]. / Abcès deltoïdien primitif a Nocardia asteroides au cours d'une maladie de Horton traitée.

In a patient treated with high doses of corticosteroids for giant cell arteritis, a renewed rise in sedimentation rate accompanied by painful limitation of mobility of the shoulder led to the discovery of an intra-deltoid fluid collection. Aspiration yielded pus, culture showing the presence of Nocardia asteroides (NA). No portal of entry nor extramuscular sites of infection were found, justifying a diagnosis of primary nocardial muscle abscess. To the best of our knowledge, this is the first reported case. The opportunity is taken to review the epidemiological and clinical characteristics of NA infections as well as new management approaches.