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Biomedical subjects

P Joly

Publications and source records attributed to P Joly.

At least 163 records · Page 9Linked to original sources

[Percutaneous extraction of a silicone catheter 5 years after its migration in the pulmonary artery].

The authors describe a case of percutaneous extraction of a piece of silicone-coated catheter, 5 years after its embolisation in the pulmonary artery and the results of electronic microscopic study of the specimen. The discussion provides the factors indicating extraction which is relatively easy with this technique irrespective of the chronicity of embolisation, in order to avoid infectious complications.

Aged↗

[Childhood acromelalgia a propos of a case revealing Fabry's disease].

The occurrence of an acrosyndrome (Raynaud's phenomenon, erythermalgia, acrodynia...) in childhood may be the first manifestation of a general disease. Though it can be an early onset Raynaud's disease, it could also be the first sign of a connective tissue disease (juvenile polyarthritis, mixed connectivitis...) or of a overload disorder. We report a case of childhood-onset acromelalgia leading to the discovery of Fabry's disease. This chromosome X-linked hereditary disorder, resulting in the ubiquitous accumulation of neutral sphingolipids, is usually rapidly suspected by the finding of "boxer-short" angiokeratoma. Diagnosis is confirmed by the ophthalmic examination (cornea verticillata), by the pathological examination of a skin sample, and by the measure of alpha-galactosidase A activity. Treatment is usually only symptomatic, but the discovery of the mutations responsible for the disease could open the way to specific therapy.

Adult↗

[Pemphigoid mimicking epidermolysis bullosa acquisita].

INTRODUCTION: Subepidermal autoimmune bullous dermatoses form a clinical entity for which there is not always an individualized clinical and pathological description. CASE REPORT: A patient presented with bullous skin disease of atypical nature. There was an almost total desepidermization of the legs, vast areas of erosion on the trunk and arms with a Nikolski sign in an area of healthy skin, buccal involvement, multiple milium cysts and ungueal dystrophies with nail loss. DISCUSSION: This clinical presentation in this patient suggested acquired bullous epidermolysis. However, according to the recently defined clinical criteria for pemphigoid, the probability of correct diagnosis of pemphigoid was greater than 95 p. 100 since nearly three fourths of the major criteria were present. This diagnosis was confirmed by reference techniques (electron microscopy, indirect electron immunomicroscopy and immunoblotting). Thus, bullous autoimmune diseases of the dermoepidermal junction can be reliably differentiated on the bases of the clinical features, together with direct and indirect immunofluorescence on salt-split skin.

Aged↗

[Allergic dermatitis caused by metallic implants in orthopedic surgery].

PURPOSE OF THE STUDY: Dermatitis due to metallic implants in orthopaedic surgery are uncommon complications and are difficult to diagnose. MATERIAL AND METHODS: A literature review found descriptions of 54 cases. All types of implants, even small in size such as screws or loops, made of stainless steel or chromium-cobalt alloys have been incriminated. There was no case related to titanium alloy. RESULTS: The clinical aspects were very different; all the forms of dermatitis are possible. The most frequent were eczemas (44 per cent), bullous dermatitis (11 per cent) and inflammatory forms (13 per cent). DISCUSSION: These dermatitis result either from immuno-allergic mechanisms or from cell-toxicity. There are two main diagnostic difficulties. 1--To confirm the responsibility of the implant in the dermatitis because the cutaneous lesions appears often generalized or localized in an area distant from the implant and because their emergence may be very belated, sometime after several years. 2--To distinguish some of these dermatitis and especially inflammatory forms, from sepsis. There is no current means to state positively this diagnosis without removing the implant. The microscopic tissues analysis surrounding the implant and the cutaneous patch-tests using fillings of the implant itself are the only methods which appear able to confirm the diagnosis. There are no reliable means to detect risk subjects, the emergence of a dermatitis seems to be unpredictable. The specificity and the sensibility of the usual patch-tests are insufficient. CONCLUSION: The main means to prevent dermatitis is to reduce the production of metallic particles due to wear, fritting and corrosion. Management is based on removing of the incriminated implant. Treatment using drugs, including local applications or general dispensing of steroids, have generally a low efficiency.

Adolescent↗

[Lymphoma with skin manifestations in HIV infection: 8 cases].

INTRODUCTION: Cutaneous lymphomas occurring in HIV infection are a rare disease. Most of them are high grade lymphomas with fulminant course and poor prognosis. OBJECTIVE: Evaluate clinical and histological aspects as well as immunophenotype and evolution of these lymphomas. PATIENTS AND METHODS: Eight patients with HIV infection were studied between 1992 and 1994. The clinical and histological features were reviewed by the members of the French Study Group for Cutaneous Lymphomas. Staging procedures for lymphomas were performed in 7/8 patients. RESULTS: Seven non epidermotropic lymphomas and one mycosis fungoides were reviewed. Patients were male (6 cases) and female (2 cases); their mean age was 45 years (27-63). The mean level of CD4 T cells/mm3 was 141 (20-380). Only one patient presented with extracutaneous lesions. These lesions were similar to seronegative patients, but unusual features were observed in two cases. Histological classification showed high grade lymphomas in 6/8 cases. The immunophenotype was: T-cell lymphoma in 4 cases, B-cell lymphoma in 3 cases; it could not be determinated in one case. Six patients died. The median of survival is 8 months in this series. DISCUSSION: Our series confirms the predominance of high grade lymphomas presenting in the skin. The T-cell phenotype is more frequent. The onset of a cutaneous lymphoma has a poor prognosis in HIV infection. Most of our patients had localised disease at presentation. Therapeutic management of these lymphomas must be codified.

Adult↗

[Melkersson-Rosenthal syndrome treated by thalidomide. 2 cases].

INTRODUCTION: Treatment of Melkersson-Rosenthal syndrome (MRS) remains uncertain. Two cases of MRS treated with thalidomide are described. CASE REPORT: Two patients with cheilitis granulomatosa and recurrent facial edema have been treated with thalidomide (100 mg daily) for 3 and 6 months respectively. The efficacy of thalidomide was complete clinically and histologically in one case. In the second one, thalidomide suppressed attacks of facial edema and reduced lip swelling. COMMENTS: These cases suggest a therapeutic effect of thalidomide in MRS.

Adult↗

Coexistent cutaneous T-cell lymphoma and B-cell malignancy. French Study Group on Cutaneous Lymphomas.

BACKGROUND: The coexistence of cutaneous T-cell lymphoma (CTCL) and a B-cell malignancy (BCM) is rare. OBJECTIVE: Our aim was to assess the clinical and pathologic aspects of coexistent CTCL and BCM and to examine potential explanations for this association. METHODS: We report six cases of concurrent CTCL and BCM in which B- and T-cell lineages were demonstrated by immunologic studies. The literature includes 13 additional cases. All 19 CTCL-BCM cases are reviewed. RESULTS: CTCL either preceded or followed the BCM, which was a low-grade malignancy in most cases (16 of 19). Possible explanations for the association include a genetic predisposition, underlying viral infection, chemotherapy-induced carcinogenesis, stimulation of a B-cell clone by malignant helper T cells, and alterations in progenitor cells before determination of B- and T-cell lineage. CONCLUSION: An alteration in progenitor cells, with subsequent oncogenic activation of variable origin, might account for most cases of coexistent CTCL and BCM.

Adult↗

Overlapping distribution of autoantibody specificities in paraneoplastic pemphigus and pemphigus vulgaris.

Paraneoplastic pemphigus is an autoimmune bullous skin disease in which autoantibodies immunoprecipitate a characteristic antigenic complex. The objective of this study was to analyze by immunoblotting and immunoelectron microscopy the autoimmune response in five patients with clinical and immunohistologic features typical of paraneoplastic pemphigus. In a first series of experiments, immunoblotting and immunoelectron microscopy were performed using anti-human whole Ig. Although immunoblotting results were consistent with the autoantibody specificities previously described in paraneoplastic pemphigus sera, immunoelectron microscopy demonstrated the presence of Ig deposits on desmosomal plaques, on hemidesmosomes and, surprisingly, on both the extracellular part of desmosomes and the keratinocyte plasma membrane. In a second series of experiments, immunoblotting and immunoelectron microscopy were carried out using antihuman IgG subclasses. The major observation was that two sera contained, in addition to the anti-desmoplakins I-II, anti-185-kD and anti-230-kD autoantibodies, autoantibodies that stained the desmoglea by indirect immunoelectron microscopy and bound to a 130-kD polypeptide by immunoblotting. One serum was particularly demonstrative: IgG1 bound to the 250- and 220-kD bands corresponding to desmoplakins I and II on immunoblots and to the desmosomal plaques of keratinocytes in immunoelectron microscopic preparations; IgG3 recognized a 185-kD immunoblotting band and hemidesmosomes and desmosomal plaques by immunoelectron microscopy; IgG4 bound to the 130-kD immunoblotting band of pemphigus vulgaris and labeled the desmoglea and the keratinocyte plasma membrane by immunoelectron microscopy. These results demonstrate that the paraneoplastic-pemphigus autoimmune response involves both intracellular and extracellular desmosomal antigens and suggest an overlapping distribution of autoantibody specificities among autoimmune bullous skin diseases.

Aged↗

Thiopronine-induced herpetiform pemphigus: report of a case studied by immunoelectron microscopy and immunoblot analysis.

We report a case of herpetiform pemphigus induced by thiopronine. Direct immunoelectron microscopy performed on peribullous skin showed IgG deposits predominantly located in the extracellular portion of desmosomes. By immunoblot analysis using bovine tongue extracts as the antigen, the patient's serum recognized a 160-kDa polypeptide which comigrated with desmoglein I. This study underlines the contribution of immunoblot analysis to the diagnosis of atypical cases of pemphigus, and confirms that herpetiform pemphigus may be a clinical variant of pemphigus foliaceus.

Aged↗

Erythema gyratum repens. A case studied with immunofluorescence, immunoelectron microscopy and immunohistochemistry.

We report a patient with erythema gyratum repens (EGR), in whom a bronchial carcinoma was found. Direct immunofluorescence revealed granular deposits of immunoglobulins at the basement membrane zone (BMZ) in the skin, and in the lung tumour. Direct immunoelectron microscopy showed that the immune deposits were localized just beneath the lamina densa. Indirect immunofluorescence revealed circulating anti-BMZ antibodies. Immunohistochemical staining, using anti-transforming growth factor-beta, anti-epidermal growth factor receptor, anti-vimentin and anti-alpha-actin, was found to be more intense in the lesional skin and the lung tumour than in normal tissues. Possible mechanisms in the pathogenesis of EGR are discussed.

Autoantibodies↗

[Spontaneous dissection of the common trunk and principal branches of the left coronary arterial system. Apropos of a case].

The authors report the case of a 45 year old woman presenting with inaugural anterior myocardial infarction due to spontaneous dissection of the left main and principal branches of the left coronary arterial system. Two attempts of thrombolysis at a one hour interval were made within 6 hours of the onset of symptoms with signs of reperfusion but the ECG and echocardiography showed anterolateral myocardial infarction. A recurrence of chest pain on the fifth day led to emergency coronary angiography. The extent and severity of the observed lesions led to emergency surgical revascularisation with no post-operative complications. Skin biopsy showed signs of dystrophy of the elastic tissues. Angiographic control ten months after surgery showed complete regression of the lesions, especially those of the left main coronary with, however, occlusion of the second segment of the left anterior descending artery and sequellae of anterior myocardial infarction. Treatment of this type of disease is difficult as the outcome is unpredictable and has to be decided case by case.

Aortic Dissection↗

[Quantitative cytological study of the activity of a new resistance modulator, S 9788, on human leukemic cells using multiparametric image analysis].

The triazinoaminopiperidine derivative S 9788 is a new multidrug resistance modulator. The modulating activity of S 9788, comparatively to those of verapamil and the combination of S 9788 and verapamil, was demonstrated on the human leukemic T cell line CCRF-CEM resistant (about 6000 fold) to vinblastine using Microculture Tetrazolium Assay. S 9788 at 5 microM, strongly potentialized the cytotoxic activity of vinblastine but the reversion of resistance remained partial. Verapamil and the combination S 9788-verapamil, tested at equimolar concentrations, were respectively 1000 and two times less active than S 9788 alone. The impact of S 9788, verapamil and their combination on the cytological modifications bound to vinblastine resistance of CEM cells was evaluated by multiparametric quantitative cytological analysis (21 nuclear parameters measured) using a SAMBA 2005 cell image processor. Treatments with the different modulators, in absence or in presence of vinblastine, had no significant effects on the morphology of sensitive CEM cells. On vinblastine resistant CEM cells, S 9788 and the combination S 9788-verapamil induced significant cytological modifications. These modifications were characterized by a partial reversion of some parameters (more specifically nuclear texture parameters) to values close to those observed in parental sensitive cells and permitted an automatic classification of these treated resistant cells in cells of "sensitive" type with a percentage superior to 50%. In conclusion, the reversion of resistance induced by S 9788 on CEM cells resistant to vinblastine does not fit only with a biological phenomenon like the efflux of cytotoxic agents but is associated with a set of cellular alterations involved in multidrug resistance.

Animals↗

[Digital necrosis of the arm excluding scleroderma. Retrospective study of 45 cases].

UNLABELLED: The causes of digital necrosis are so diverse that it should be considered not as a surgical but as a medical emergency. In women, digital necrosis first suggests connective tissue disease, and especially Progressive Systemic Sclerosis. In men the causes are more diverse. In this retrospective study, we have tried to identify and categorize the cause of digital necrosis in patients without Progressive Systemic Sclerosis. The medical files of all patients admitted from 1/1/89 to 1/1/93 for digital necrosis were retrospectively reviewed. All patients with Progressive Systemic Sclerosis, either diagnosed at that time or know before, were excluded. All 45 patients in the study had an evaluation of coagulation, vascular risk factors, a cardiac examination and nailfold capillary microscopy, as well as tests for antinuclear antibodies, and cryoglobulinaemia. Other tests were done according to the clinical presentation. Upper limb angiography was done 26 times. RESULTS: there were 10 women and 35 men, mean age 53 years (24-85 years). In the women, the following diagnosis were found: atherosclerosis 1, paraneoplastic syndrome 1, one thrombocythemia, and 6 connective tissue diseases (one dermatopolymyositis, 1 essential cryoglobulinaemia, 2 lupus erythematous and two suspected secondary Raynaud's phenomenon. Twelve men had arterial diseases: 6 thromboangiitis obliterans and 6 obliterans atherosclerosis. Seven times, the cause was local: 5 hammer's syndromes, 2 thoracic outlet syndrome. In the other patients, the causes were extremely diverse: cardiac embolism (4), primary antiphospholipid syndrome (3), polyarteritis nodosa (1), leprosy seroconversion (1), connective tissue disease (3). In 4 patients (3 of whom were smokers) no cause was found.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Protein S deficiency in necrotizing cellulitis of the penis].

INTRODUCTION: The pathogenesis of necrotizing cellulitis remains uncertain. Factors that enhance clot formation may contribute to tissue necrosis. CASE REPORT: A case of necrotizing cellulitis of the penis in a patient with a transient protein S deficiency, is reported. CONCLUSION: The responsibility of this transient hypercoagulable state in the occurrence of tissue necrosis in this patient is discussed.

Adult↗