[Evaluation of the activities of several erythrocyte enzymes (G6PDH, 6PGDH, PHI, aldolase and LDH) in psoriasis].
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Biomedical subjects
Publications and source records attributed to R Laurent.
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INTRODUCTION: Adult-onset Still's disease (AOSD) is a systemic affection characterized by intermittent fever, evanescent rash, polyarthralgia or arthritis and neutrophilic leucokytosis. We report a new case of AOSD with persistent pigmented plaques in a Vietnamese woman. CASE REPORT: A 23 year-old woman had presented within several months: inflammatory polyarthralgia, intermittent fever, asthenia, myalgia, weigh loss, polyadenopathy and an evanescent cutaneous rash. In addition, she presented pigmented skin plaques on the trunk. The investigations noted: neutrophilia (10000/mm3) and a high level of serum ferritin (42,000 microg/l). The erythrocyte sedimentation rate was 55mm in the first hour. C reactive protein was normal. HIV and syphilis serologies, antinuclear antibodies and rheumatoid factor were negative. No growth of micro-organisms was observed in repeated blood and urine cultures. The histology of the persistent plaque and lymph node were not specific. It required a high dose of corticosteroid to obtain remission. DISCUSSION: The existence of persistent plaques in AOSD is uncommon. Nevertheless, the exact relationship between the fixed cutaneous lesions and AOSD remains unclear. But their synchronous evolution with systemic symptoms and response to therapy suggest that they represent a specific manifestation of the disease.
AIMS AND BACKGROUND: The reproducibility of histologic diagnoses, and in particular of the distinction between basal and squamous cell forms, has been evaluated in the framework of a multicenter case-control study (in Italy, France, Spain and Switzerland) aimed to assess the causes of non-melanocytic skin cancers. METHODS: A panel composed of 10 pathologists from the collaborating centers was appointed. A total of 1,774 slides of routine diagnoses were blindly reviewed by a second panelist; discordant diagnoses underwent a third examination. Controversial and interesting cases were discussed during general sessions. RESULTS: The results showed a high degree of concordance (99.5%), with a Cohen's kappa of 0.85 (95% CI, 0.77-0.94) in the assessment of malignancy of lesions. The concordance in the distinction between large morphologic groups, basal cell carcinoma and squamous cell carcinoma was also high (Cohen's kappa = 0.85; 95% CI, 0.82-0.89). The assessment of histologic subtypes, degree of invasion and differentiation showed a lower degree of concordance, presumably as a consequence of a weaker consistency in the relevant classifications. CONCLUSIONS: The reproducibility study has therefore validated the epidemiologic study and in particular allowed to correct some misclassifications that could have lessened the analysis of the case-control study. In general, because of its characteristics (number of pathologists and variety of their origins, the large number of cases examined, blind examination of histologic slides), the conclusions of the study may show a certain degree of generalization, at least with regard to the countries represented. Routine histologic diagnoses of cutaneous carcinoma showed a high degree of reliability with reference to the assessment of malignancy and the distinction between basal and squamous cell carcinoma morphotypes.
BACKGROUND: We report two cases of generalized granuloma annulare occurring in photoexposed areas in two liver transplant recipients. CASE REPORTS: Case 1 was a 65-year-old man who had undergone liver transplantation in 1992. He was given immunosuppressive and antihypertensive therapy. Within 18 months of transplantation, he developed a confluent rash with maculae on sun-exposed areas (neck opening, nape, arms) and sparing the undershirt area. Clinical examination was normal. Skin biopsy revealed a palissade infiltrate located in the middle and upper derma, suggestive of granuloma annulare, with elastophagocytosis patterns (orcein stain and ultrastructure study). Photoexposure granuloma was diagnosed. Case 2 was a 59-year-old man who had undergone liver tranplantation in 1994. He was given immunosuppressive and antihypertensive therapy and developed within 4 months a dark rash on the neck opening and nape, sparing photoprotected areas. Histopathology revealed granuloma annulare. Elastophagocytosis was disclosed by orcein stain and the ultrastructure study. DISCUSSION: This clinical presentation of granuloma annulare in two liver transplant recipients is unusual. We discuss the clinical and histopathological patterns observed in our two cases and the relationships between granuloma annulare and immunosuppression.
BACKGROUND: The association between mucosal oncogenic human papillomaviruses (HPV) and bowenoid papulosis or genital Bowen's disease is well documented. In contrast this association with extra-genital Bowen's disease is poorly studied. The aim of this study was to detect oncogenic (16/18, 31/33/51) and non oncogenic (8/11) mucosal HPV using a in situ hybridization method in 28 skin biopsy specimens of extra-genital Bowen's disease. PATIENTS AND METHODS: Twenty-eight cases of extra-genital Bowen's disease seen in the period 1990-96 in the Dermatology department were included: 19 women and 9 men (mean age: 72 years). Bowen's disease locations were: hands and feet (8 cases), limbs (11 cases), face (8 cases), trunk (1 case). Blinded histopathologic examination confirmed the diagnosis of Bowen's disease and signs of HPV infection (koilocytosis). In situ hybridization was performed using three biotinylated probes detecting HPV types 6/11, 16/18, 31/33/51. RESULTS: Oncogenic HPV genoma was detected in 8 skin samples (28.6 p. 100). In all these cases, 16/18 probe was positive and in two cases, both 16/18 and 31/33/51 probes were positive; 4/8 Bowen's diseases of the extremities were positive for HPV. Koilocytes were found in 6/8 of skin samples with positive HPV detection. DISCUSSION: Mucosal oncogenic HPV are detected by in situ hybridization in 28.6 p. 100 of extra-genital Bowen's disease. In situ hybridization is an easier technique than Southern-Blot hybridization which is the gold standard. Five studies reported similar results and three studies reported different results that we discuss. A precise understanding of oncogenic HPV implication in the development of extra-genital Bowen's disease could lead to the development of new therapeutic strategies (topical cidofovir or imiquimod).
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About a recent case of acrogeria a review of the 18 cases of the litterature defines the clinical and evolutive characteristics of this primary atrophy of the skin. The histopathological and ultrastructural study of the pathological dermis with regard to a reference specimen of same age and sex shows fibroblastic and fibrillary alterations. The hyperplastic orceinophilic material is mainly of the "pseudoelastic" type. The collagenous fibers are hypoplastic and often immature. An abundant granulofilamentous substance is visible in the extracellular space and in the fibroblasts proper; it is often intricated with the pseudoelastine and contains immature collagenous fibers. The cytoplasm of the fibroblasts is swollen by ergastoplasmic vacuoles filled with the granulofilamentous substance and contains numerous pseudomyelinic bodies. All these anomalies suggest that acrogeria could be a disease of the fibroblast.
High risk human papillomaviruses (HPVs) have emerged as risk factors for anal carcinoma, of which incidence is higher in HIV-positive patients than in the general population. The aim of our study was to investigate the prevalence and risk factors for anal HPV infections in HIV-positive patients with or without history of anal intercourse. Fifty HIV 1-infected patients (36 men and 14 women) were tested at entry and followed-up every 3 months for one year for the presence of anal HPV DNA by the Hybrid Capture II trade mark assay. A series of 50 HIV-negative subjects matched for age and sex served as controls. At enrollment, anal HPV DNA was present in 29/50 HIV-positive patients (58 %) and in 3/50 control subjects (6 %). High risk (HR) HPV genotypes were detected in 20/50 HIV-positive patients (40 %) with no difference in homosexual men and other HIV-positive patients. Risk factors for HPV infection were CD4 + cell counts less than 500/microL (RR: 2.13 [95 % CI: 1.0-4.7]) and history of anogenital warts (RR: 2.36 [95 % CI: 1.2-4.6]). The HPV load was higher in patients with CD4+ < or = 500/microL than in patients with CD4 + > 500/microL (p < 0.04). During the follow-up, anal HR HPV DNA was repeatedly identified at high levels in 5 HIV-positive patients. There is some convincing evidence that HIV-positive patients with low CD4+ cells, whatever the routes of HIV transmission, have a high rate of anal HPV infection and might be at increased risk of developing anal neoplastic lesions. Identifying HR HPV infection might be warranted in immunosuppressed patients.
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Chronic venous insufficiency (CVI) of the lower limbs is a complex and fluctuating disease by its pathogenic mechanisms and its clinical symptoms. Although symptoms are subjective, they affect the quality of life and socio-professional activity of many patients. This is why convincing demonstration of therapeutic activity of a venotropic drug should be carried out according to strict methodology. Only randomized double-blind controlled trials versus placebo (no reference drugs being available) could demonstrate the activity on condition that they are set up in a protocol, the statistical design of which, is adapted and defined "a priori". Inclusion, non inclusion and judgement criteria must be rigorous, taking into account many exogenous and endogenous factors which could have influence on the severity or the change in CVI and on the comparability of groups at the beginning and at the end of the study. Thus, the main risk factors of CVI (heredity, obesity, obstetrical and gynecological history, estroprogestogen treatment, profession, environment, etc.) and the season when the patient is recruited should be taken into account. With respect to all these restraints on methodology, the venotrotopic activity of a flavonoid Daflon 500 mg (2 tablets daily) was demonstrated in 200 patients (174 women, 26 men) with organic CVI (n = 83) or functional CVI (n = 117) treated for two months in two double-blind randomized trials versus placebo. The venotropic activity of Daflon 500 mg, was shown by a significant reduction of CVI signs and symptoms, whether organic or functional, and a significant improvement in venous hemodynamics according to plethysmographic parameters. Good acceptability was observed after medium and long term trials.
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Although the association of Sweet's syndrome with haemoproliferative disorders or solid malignant tumors is well known, only two previous observations associated with T lymphoma have been reported. We report the first observation of Sweet's syndrome associated with cutaneous T cell lymphoma. The role of a cytokine cascade released by an abnormal T cell clone is discussed as an initiating event responsible for secondary infiltration with neutrophils.
Since 1987, about 60 cases of porphyria cutanea tarda (PCT) associated with human immunodeficiency virus (HIV) have been reported. The respective roles of HIV and toxic hepatic factor in PCT remain unclear. We report 10 new cases and analyse the following toxic hepatic factors: hepatitis C and B, alcoholism, drugs. The route of HIV transmission to these 10 men were: IV drugs abuse (3), homo/bisexuality (4), heterosexuality (1), and unknown (2). When PCT was diagnosed, their average age was 38 years (29-54) and the HIV-infection had been established for 4.8 years (0.33-9). Seven men had HIV-related symptoms and a CD4+ lymphocyte count below 200/mm3. Cutaneous signs and urinary porphyrin count were characteristic. Alcohol abuse was present in 8/10 patients. AST, ALT and/or gamma GT were high in 9/10 patients; 5/10 patients had HCV antibodies (4 were HCV-PCR positive). HBs antigenemia was negative among the 5/8 patients with HBV antibodies; 10/10 patients took prescribed hepatotoxic drugs. Our series confirms the presence of toxic hepatic factors in PCT of HIV-positive patients. Hepatitis C, alcoholism and hepatotoxic drug consumption seem to be triggers for the appearance of PCT in HIV-positive patients.