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Biomedical subjects

P Joly

Publications and source records attributed to P Joly.

At least 55 records · Page 3Linked to original sources

Scleroderma-like cutaneous lesions induced by paclitaxel: a case study.

Paclitaxel is a recent antineoplastic agent that belongs to the taxane family. Its activity has been demonstrated in advanced and refractory ovarian, breast, lung, and head and neck cancer. Adverse cutaneous reactions to paclitaxel have been reported, namely bullous fixed drug eruption, onycholysis, acral erythema, erythema multiforme, and pustular eruption. We report the first case of scleroderma-like changes after paclitaxel administration. A 63-year-old patient presented with an edematous and infiltrated erythema of the head, neck, axillae, and left hand 10 days after administration of paclitaxel and paraplatin for primitive peritoneal cancer. Cutaneous lesions improved after a change from paclitaxel to cyclophosphamide. Cutaneous lesions recurred 3 months later, after reintroduction of paclitaxel, and progressively evolved to cutaneous sclerosis. Skin biopsy showed a dermal fibrosis. Biologic tests revealed no autoimmunity. Scleroderma-like lesions of this patient were reminiscent of previously reported cases that occurred after administration of docetaxel, which also belongs to the taxan family. Thus, scleroderma-like syndromes seem to represent a unique cutaneous adverse event caused by taxanes.

Antineoplastic Agents, Phytogenic↗

[Treatment of pemphigus vulgaris by azathioprine and low doses of prednisone (Lever scheme)].

INTRODUCTION: The so-called "Lever scheme" therapeutic regimen has been proposed in the borderline forms of pemphigus to reduce the side effects of systemic corticosteroids. PATIENTS AND METHODS: A retrospective study was conducted in 8 hospital centers. The criteria for inclusion were the clinical diagnosis of pemphigus, confirmed by histological examination and direct immunofluorescence and first line therapy using the "Lever scheme" protocol, combining 40 mg of prednisone on alternate days and 100 mg/day of azathioprine. RESULTS: Twenty-two patients, seen between January 1990 and December 2000 were included in the study. Eighteen patients (82 p. 100) exhibited complete healing of their cutaneous-buccal lesions after a mean delay of 4.3 months. The lesions of 4 patients did not heal. Three of these patients died: a bed-ridden patient, a patient exhibiting a metastatic bronchial carcinoma and a hypertensive patient who died following a hemorrhagic cerebral vascular accident. Twelve patients (54 p. 100) were weaned off treatment after a mean duration of 2.9 years. Five severe adverse events were observed: one pneumonia, 2 unbalanced diabetes, one hepatitis and one pulmonary embolism. DISCUSSION: This study showed that the healing of the cutaneous-buccal lesions was obtained using the "Lever scheme" in 18 cases out of 22 (82 p. 100). The delay to healing was relatively long in view of the delayed effect of azathioprine. This limits the use of the "Lever scheme" protocol to non-extensive and/or early stage pemphigus. The severe adverse events occurred in low-weight patients in whom the dose related to weight was the highest. Hence the doses of azathioprine and prednisone should be adapted to patients' body weight.

Adult↗

[Assessment off-label prescribing in Dermatology].

INTRODUCTION: The official policy of the French National Health Insurance system is to deny reimbursement for drugs prescribed for off-label indications. The objectives of this study were 1) to quantify the use of off-label prescriptions by physicians from a hospital department of dermatology in France; 2) to characterize these off-label prescriptions; 3) to assess data from the literature on the appropriateness of these off-label prescriptions. PATIENTS AND METHODS: For each patient consulting between February 1 and April 1, 2001, the symptom or the disease that was treated and the type of prescription were recorded on standard forms. RESULTS: Eighty six percent of prescriptions were labelled, 14 p. 100 were off-labelled. Inflammatory and hypersensitivity dermatoses were the most frequent indications of off-label prescriptions (26 p. 100). Treatments which most frequently corresponded to off-label prescriptions were topical corticosteroids and methotrexate. Examination of the literature showed that 70 p. 100 of the off-label prescriptions were not based on strong data from evidence-based-medicine. Many off-label prescriptions were made by the most graduate physicians. DISCUSSION: This study showed a great number of off-label prescriptions in dermatology. These prescriptions were often related to rare diseases that were managed by senior dermatologists. These off-label prescriptions were rarely in accordance with data from evidence-based-medicine.

Decision Making↗

[Treatment of erosive oral lichen planus with thalidomide].

INTRODUCTION: Treatment of erosive oral lichen planus is difficult and often requires the use of systemic corticosteroids. This severe condition may lead to weight loss and impairment of patients' general condition due to painful oral erosions. The aim of this study was to evaluate the usefulness of thalidomide in the treatment of severe erosive oral lichen planus. PATIENTS AND METHODS: The efficacy and safety of thalidomide were retrospectively evaluated in 6 patients with severe erosive lichen planus resistant or relapsing despite high doses of oral corticosteroids. Thalidomide was started at an initial dose of 50 to 100 mg/day and was then progressively decreased to the minimal effective dose. Follow-up evaluations were performed every two months by the same dermatologist. RESULTS: Complete healing of erosive lesions was observed in 4 of 6 patients after a mean duration of 4 months. Partial epithelialization of erosive lesions, disappearance of dysphagia and weight gain were observed in one patient. Treatment failed in the last patient. The mean dose of prednisone of the 3 patients receiving both thalidomide and oral corticosteroids decreased from 37 mg/day at the beginning of the study to 7 mg/day at the end of the study. Two patients experienced severe side effects: phlebitis and neuropathy, leading to thalidomide discontinuation. Oral erosions rapidly relapsed after withdrawal of thalidomide. DISCUSSION: Thalidomide seems to be an effective treatment of severe corticosteroid resistant and dependent or when systemic corticosteroids are contraindicated erosive oral lichen planus. Potentially serious side effects should restrict its use to the most severe forms of the disease.

Aged↗

Tadpoles' responses to risk of fish introduction.

The introduction of predatory species, such as fish, in amphibian breeding sites is one of the many likely causes of amphibian population decline. The existence of inducible or constitutive (permanent) defences is expected to temper the lethal effects of fish on tadpoles. According to current theories on the evolution of phenotypic plasticity, the amphibian species that occur in habitats that are mostly or often fish-free were expected to display inducible defences, while the species that reproduce mainly in fish ponds ought to present constitutive defences. The tested species were Rana dalmatina, Hyla arborea and R. ridibunda, respectively. As expected, R. ridibunda tadpoles were less vulnerable to fish predation than the other species. Nevertheless, all three species exhibited morphological responses in the presence of fish. Their increases in tail area and decreases in body depth were similar. However, behaviour was only modified in R. dalmatina, which exhibited lower activity rates and higher refuge use in the presence of fish cues. Such lower activity rates were related to lower growth and developmental rates. While not detected in this experiment, the existence of inducible defences in the three species suggests the existence of costs of anti-predator defence. Those costs may have delayed effects so that increasing the frequency of fish ponds may exacerbate the impact of delayed costs whatever the species, suggesting that the dynamics of species that are not fish-adverse could also suffer from the introduction of fish.

Adaptation, Physiological↗

Pemphigus is not associated with allotypic markers of immunoglobulin kappa.

The kappa light chain constant region of immunoglobulins bears polymorphic markers involved in susceptibility to various autoimmune diseases. To determine whether it also contributes to the occurrence of pemphigus, a group of autoimmune blistering skin diseases owing to pathogenic autoantibodies, the genotypic frequencies of Km allotypes were evaluated in patients with pemphigus foliaceus or pemphigus vulgaris and ethnically-matched healthy controls in both Tunisia and France. No difference in the distribution of Km genotype or allele frequencies was observed between patients and controls in either countries. Therefore, Km allotypes do not appear to constitute a genetic factor contributing to pemphigus.

Adult↗

Epistasis between DSG1 and HLA class II genes in pemphigus foliaceus.

Pemphigus foliaceus (PF) is a rare and severe cutaneous autoimmune disease caused by autoantibodies directed against desmoglein 1 (DSG1), a desmosomal adhesion glycoprotein. We previously showed that the DSG1 gene is polymorphic and that a coding synonymous T/C single nucleotide polymorphism at position 809 is associated with PF. To determine whether the disease occurred as a consequence of complex genetic interactions, we simultaneously examined the contribution of major histocompatibility complex (MHC) class II and DSG1 polymorphisms to PF susceptibility. Our analysis performed in 31 PF patients and 84 healthy controls first confirmed the previously reported common DRB1*04 and DRB1*14 genetic background in PF and individualized DRB1*0102, DRB1*0402 and DRB1*0406, and DRB1*1404 as susceptibility MHC class II alleles in French Caucasian PF patients. It also showed that the C/C(809) genotype was associated with PF. Combined analysis of HLA class II and DSG1 polymorphisms with several distinct statistical methods including logistic regression, showed that the DRB1*04 allele and the C/C(809) genotype interact to confer a higher susceptibility to PF. These data demonstrate the role of epistasis between individual genes in PF susceptibility and illustrate the genetic complexity of organ-specific autoimmune diseases.

Autoantigens↗

[Chronic eczematiform eruption in the elderly].

INTRODUCTION: Eczematiform eruptions in the elderly represent a relatively frequent motive for consultation and may lead to repeated hospitalization. Their etiologic diagnosis is often difficult and explains the frequent relapses. The frequent relapses can be explained by the difficulty in determining their etiologic diagnosis. The aims of this study were: 1) to specify the evolving characteristics of these eruptions in elderly patients and 2) determine their etiology. PATIENTS AND METHODS: The inclusion criteria in this retrospective study were: patients aged over 65 at the time of diagnosis, presenting with extensive eczematiform eruption (> 20 p. 100 of body surface) and lasting for more than one month. Eczema on stasis dermatitis of the lower limbs and generalized contact eczema were excluded. Eighty-three patients followed between January 1990 and January 1999 were included. The clinical, biological, histological and evolving characteristics were analyzed. RESULTS: Mean age of patients was 77 +/- 8 years and the male female sex ratio was 2.4. Patients received a mean of 4.0 +/- 2.6 drugs/patient, consisting essentially of cardiovascular and psychotropic agents. The cutaneous eruption had evolved a mean of 12.5 months (1 to 48 months) before diagnosis. Eczema was pruriginous in 92 p. 100 of cases. Frequent relapses were observed in 68 p. 100 of cases. Precise etiologic diagnosis was retained in 48 patients (58 p. 100). This was disseminated contact eczema (n=19), lymphoma cutis (n=10), atopic eczema (n=7), scabies acariasis (n=6) and pemphigoid (n=6). No etiologic diagnosis was retained in the remaining 35 patients (42 p. 100). Comparison of the characteristics in the 2 groups showed excessive consummation of medicinal products (p=0.024), predominant eruption of sun-exposed areas (p=0.004) and a greater frequency of histological images of keratinocyte necrosis (p=0.0072) in patients presenting eczematiform eruptions of unknown etiology. DISCUSSION: These observations suggest the eventual responsibility of medicinal products in the occurrence of certain extensive and chronic eczematiform eruptions in the elderly. However, the delays of imputability of various causal drugs were often longer than those currently admitted for toxidermia, and the withdrawal of potentially imputable agents rarely led to spectacular improvement in the lesions. A case test report is in progress to specify this hypothesis.

Aged↗

[Bullous pemphigoid induced by spironolactone].

INTRODUCTION: The role of spironolactone as pemphigoid-inducing agent has recently been suggested. We report a new case of pemphigoid probably induced by spironolactone. The disease resolved after withdrawal of the diuretic drug containing aldactone. OBSERVATION: A 76 year-old patient was hospitalized for eczematiform lesions and severe pruritus. Histological and immunological investigations led to the diagnosis of pemphigoid. For several years, the patient had been treated with acarbose, amlodipine, fluvastatine, buflomedil, lysine acetylsalicylate and a spironolactone-furosemide association. On withdrawal of spironolactone alone, the cutaneous lesions regressed spontaneously within 15 days and no relapse was noted 30 months later. DISCUSSION: Numerous observations have suggested the role of certain drugs in the occurrence of pemphigoid. A recent test case study concerning drugs administered to patients suffering from pemphigoid has shown the significant association with spironolactone and neuroleptics. In our case report, the imputability of spironolactone is plausible. Because of the poor prognosis of pemphigoid, essentially due to iatrogenic complications, it is valuable to be able to delay the initiation of specific treatment for a few days, in patients receiving spironolactone.

Aged↗

[Aneurysm and axillary crutches].

Axillary crutches may injure the axillary artery. A 65-year-old woman with a leg length discrepancy of 30 cm subsequent to childhood poliomyelitis was seen for subacute ischemia of the right upper limb. Axillary aneurysm with thrombosis due to chronic use of axillary crutches was diagnosed after clinical examinations and surgery. Resection-graft of the aneurysm and thrombectomy was performed and restored patency. Axillary crutches may cause arterial stenosis or aneurysms. Aneurysms may be complicated by acute thrombosis, chronic or acute distal embolization, or axillary mass formation. Besides not using axillary crutches, surgical treatment of crutch-induced axillary aneurysm must be performed before sometimes definitive sequelae develop.

Aged↗

[Pregnancy: a possible etiology of pyoderma gangrenosum. A case report and review of the literature].

OBJECTIVE: We report an unusual case of pyoderma gangrenosum at 26 weeks gestation and review other cases reported in the literature in order to examine the underlying causes and determine the appropriate diagnostic and therapeutic approach to this uncommon skin disease. Patient and methods. The patient developed an acute abdominal syndrome at 26 weeks gestation (laparotomy was non contributive) followed by pyoderma gangrenosum of the abdominal wall. Seven other cases have been reported in the literature associating pyoderma gangrenosum with pregnancy. We analyzed the clinical findings in these cases. RESULTS: Corticosteroid therapy successfully controlled pyoderma gangrenosum in our patient. Pregnancy outcome was favorable with little effect on the newborn. Complete healing was achieved after several months of treatment. CONCLUSION: Histological lesions are nonspecific. Diagnosis of pyoderma gangrenosum is generally based on clinical signs. Systemic disease is associated in half the cases (inflammatory bowel disease, myelodysplastic syndrome, rheumatoid arthritis). The impact on pregnancy appears to be limited. Treatment is based on high-dose systemic corticosteroid therapy or cyclosporin. The two drugs may have to be combined. Search for an associated disease and appropriate treatment is particularly important for the outcome of pregnancy.

Abdominal Wall↗

[Acute vulval ulcer or Lipschutz's disease].

The case of a 16-year-old woman with typical clinical features of acute vulval ulcer, Lipschutz's disease, is reported. The main differential diagnoses are discussed. Isolated vulval ulcer can reveal a recurrent aphthous ulcer, Behçet's disease or Crohn's disease. In contrast, acute vulval ulcer spontaneously heals with no recurrence, that must be taken into account for diagnosis.

Acute Disease↗

[Survey on the activity of hospital departments of dermatology in France].

INTRODUCTION: The Collège des Enseignants de Dermatologie de France initiated a study to assess the activity of the departments of dermatology in French hospitals. We report the results of this survey. MATERIAL AND METHODS: The study was conducted during a randomly selected week in the year 2000. An anonymous questionnaire was sent to all the departments of dermatology in the hospitals in France and was completed for each patient leaving the hospital during that week. The data collected were: the type of establishment, admission method, hospitalization method, type of pathology, motive for hospitalization, length of hospitalization and type of treatment used. RESULTS: The response rate was of 96 p. 100 for the University hospital centers and 80 p. 100 for the non-university hospitals. The number of patients treated per year was of 65 628 for the University hospitals and of 16,000 for the others. Ninety percent of the hospitalizations corresponded to direct admissions. The pathologies at the origin of the majority of yearly hospitalizations were, for the University hospitals: melanoma, leg ulcers, highly specialized dermatoses and systemic diseases and for the other hospitals: leg ulcers, HIV infection, highly specialized dermatoses and erysipela. The pathologies requiring the greatest number of hospitalization days were melanoma, leg ulcers, erysipela and highly specialized dermatoses. DISCUSSION: This study shows that the activity in the departments of dermatology is centered on pathologies that are clearly severe in terms of Public Health (oncology, infectious diseases and angiology). It also shows the efforts made for the management of patients in out-patient settings.

Delivery of Health Care↗

Mortality with dementia: results from a French prospective community-based cohort.

Despite the magnitude of the problem, little is known about the duration of dementia. Survival and risk factors of mortality with dementia and the impact of dementia on the risk of death were investigated using the Personnes Agées Quid (PAQUID) prospective population-based cohort study between 1988 and 1998. Statistical models dealing with interval censoring were performed. Among 3,675 participants aged 65 years or older and initially nondemented, 2,923 have been followed up for 8 years. Of these, 281 persons with incident dementia were actively diagnosed. The mean age of onset of dementia was 82.3 years. In the total population, the relative risk of dying after developing dementia was estimated to be 1.82 (95% confidence interval (CI): 1.77, 2.68) when adjusted for sociodemographic variables and comorbidity. Deaths from cerebrovascular diseases and respiratory diseases were particularly increased among persons with dementia, compared with those without. The median survival time of the persons with dementia was estimated to be 4.5 years. Women with dementia had a longer survival than did men with dementia, particularly for Alzheimer-type dementia (relative risk = 0.47, 95% CI: 0.27, 0.83). Educational level was not significantly associated with survival in persons with dementia. These results provide further evidence of the malignancy of dementia, which will be a challenge for the 21st century.

Age of Onset↗