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Biomedical subjects

J Revuz

Publications and source records attributed to J Revuz.

At least 91 records · Page 5Linked to original sources
PubMed1999

The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid.

Seven patients with mild pemphigus vulgaris (n = 3) or pemphigus foliaceus (n = 4) were treated with a very potent topical corticosteroid alone. Clobetasol propionate 0.05% cream was applied to mucosal lesions and involved skin twice a day for at least 15 days, then progressively tapered. Pemphigus was considered to be controlled if healing of lesions was obtained, with a 75% decrease in the number of new lesions per week without addition of any systemic treatment. In all seven patients, the disease was controlled initially with healing of cutaneous lesions within 15 days, while healing of mucosal lesions took at least 1 month. In four patients, remission was maintained with topical corticosteroid alone for a mean 19-month follow-up. In three patients, relapse occurred after 2-11 months, requiring a systemic treatment.

Administration, Topical↗
PubMed1999

Neurological complications of neurofibromatosis type 1 in adulthood.

Neurofibromatosis type 1 (NF1) is a genetic disease with a wide range of neurological manifestations. To examine these, and to evaluate neurological morbidity in adulthood of patients with NF1, we studied a hospital-based series of 158 patients that included 138 adult patients aged >18 years and 20 children. NF1 evaluation included a multidisciplinary clinical and a clinically oriented radiological investigation. Neurological events occurring during childhood (in both children and adults of the series) and adulthood were recorded. One or several neurological manifestations have been observed in 55% of patients (adults and children) (n = 87). These included: headache (28 patients); hydrocephalus (7); epilepsy (5); lacunar stroke (1); white matter disease (1); intraspinal neurofibroma (3); facial palsy (1); radiculopathy (5); and polyneuropathy (2). Tumours included: optic pathway tumours (20); meningioma (2); cerebral glioma (3); and malignant peripheral nerve sheath tumours (6). Life-threatening complications were observed in five adults and included four malignant peripheral nerve sheath tumours and one meningioma. Pain was the leading symptom in 11 adults and was related to malignant peripheral nerve sheath tumours, complications of intraspinal neurofibromas, subcutaneous neurofibromas and peripheral nerve neurofibromas. NF1 in adults was not associated with other disabling or life-threatening neurological complications. Symptomatic optic pathway tumours, cerebral gliomas, symptomatic aqueductal stenosis and spinal compression due to intraspinal NF were observed exclusively during childhood. In this series, the predominant neurological features of adults with NF1 were chronic pain and malignant peripheral nerve sheath tumours.

Adolescent↗
PubMed1999

Comparative analysis of severe aphthosis and Behçet's disease: 104 cases.

BACKGROUND: The nosologic field of aphthosis is not well defined. While criteria for Behçet's disease (BD) are lacking in severe unipolar or bipolar aphthosis, these disorders however lead to an important impairment of quality of life. The aim of this study was to assess the relationship between severe aphthosis and BD. METHODS: Records of all patients hospitalized for aphthosis or BD during a 20-year period were reviewed. RESULTS: According to clinical data, cases (n = 104) were classified as unipolar aphthosis (group I, n = 53), bipolar aphthosis (group II, n = 30) or BD (group III, n = 21). The characteristics of aphthae (number, localization, recurrences, presence of scars) were similar in all groups. Specific cutaneous manifestations (erythema nodosum, pseudofolliculitis, papulonodules) were present in 15, 17 and 95% of patients of groups I, II and III. Cutaneous puncture hypersensitivity was present in 14 out of 18 patients of group III and no patient of the two other groups. At least one extramucous specific manifestation (cutaneous, ophthalmologic, neurologic, articular, vascular) was present in 43% of patients of group I and II, and 100% in group III. CONCLUSION: These data support the hypothesis of a continuous spectrum linking severe aphthosis to BD.

Adolescent↗
PubMed1999

Increased fibroblast elastase activity in acquired cutis laxa.

BACKGROUND: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity. OBSERVATION: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and from healthy controls. Histology showed only few perivascular lymphocytes in lax ear skin and a dense inflammatory infiltrate in urticarial skin. In both biopsies elastic fibres were decreased as demonstrated by computerized morphometric analyses. Elastase activities of fibroblasts in culture were evaluated. There was a 2- to 3-fold increase in elastase activity in urticarial skin fibroblasts, contrasting with a normal elastase activity in lax ear skin. CONCLUSION: Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.

Adult↗
PubMed1999

Optimising the use of tazarotene in clinical practice: consensus statement from the European advisory panel for tazarotene (Zorac TM).

BACKGROUND: This paper reports the proceedings of the European Advisory Panel Meeting for tazarotene (Zoractrade mark), which took place in Cologne on May 7, 1998. AIM: The aim of this meeting was to discuss recommendations for the use of tazarotene based on the clinical data available and on the clinical experience of the Advisory Panel members, and to identify future research needs. RECOMMENDATIONS: Based on currently available data, tazarotene can be used for the treatment of chronic, stable, plaque-type psoriasis, on the trunk or limbs covering up to 20% of the body surface area. In clinical trials, patients generally experienced a clinical response within 4 weeks of starting tazarotene treatment, and improvement was maintained for up to 12 weeks after stopping therapy. Results from published and not yet published clinical trials show that the efficacy and tolerability of tazarotene can be enhanced by the addition of topical corticosteroids to the treatment regimen and that, when used in combination with broad-band UVB phototherapy, tazarotene reduces the amount of UV light required to treat plaques. Tazarotene gel is available in two concentrations, 0.05 and 0.1%. The Advisory Panel recommends that the choice of concentration should be based on factors such as the irritability of the patient's skin and the thickness of plaques. Irritation can be managed by reducing the concentration or frequency of application, or by adding a topical corticosteroid to therapy. Tazarotene shows promise as a treatment for psoriasis in special localisations, such as the scalp, face and skin folds, although clinical studies are required.

Humans↗
PubMed1999

[Lyell syndrome and Stevens-Johnson syndrome caused by lamotrigine].

BACKGROUND: Lamotrigine is a new anticonvulsant belonging to the triazine family. Several cases of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) have been described in patients taking this drug. We report 2 cases in children attending the same hospital. CASE REPORTS: Two children, aged 9 and 13 years, developed SJS and TEN respectively, 3 and 28 days after lamotrigine was added to their usual anticonvulsant regimen. In both cases, outcome was favorable despite major decline in psychomotor capacity in one. In the first case, chronological attributability was plausible for lamotrigine and doubtful for sodium valproate, clonazepam and hydrocortisone. In the second case, chronological attributability was probable for amoxicillin, plausible for lamotrigine and doubtful for sodium valproate, but the numerous previous absorptions of amoxicillin made lamotrigine more suspect. DISCUSSION: The risk of Steven-Johnson syndrome and toxic epidermal necrolysis is high with lamotrigine with an estimated frequency of 1/1000. This risk is probably higher than with other anticonvulsants. Associating lamotrigine with sodium valproate increases the frequency of adverse skin reactions.

Adolescent↗
PubMed1999

[Symptomatology and markers of the severity of erythematous drug eruptions].

OBJECTIVES: Skin reactions to drugs affect about 2.2 percent of inpatients. More often, they are described as morbilliform or maculopapular. The objectives of this study were to characterize these skin reactions and to look for severity markers. METHODS: We retrospectively analyzed 133 cases of drug reactions collected in a Dermatology unit from 1973 to 1995 for whom, photographs and blood cell count were available. Well recognized disorders were excluded (Stevens-Johnson syndrome, Lyell syndrome, TEN, AGEP, vasculitis, phototoxicity, fixed drug eruption). Severity was defined on 3 criteria: hospitalization, skin reaction prolonged more than 21 days and visceral involvement. For each criteria, we studied the association with clinical and laboratory findings, first with univariate analysis, then with multivariate analysis for significant associations. RESULTS: Characteristics of the 133 included cases were: women: 58 p. 100, mean age: 52 years, inpatients: 80 p. 100, mean interval from beginning of drug therapy to reaction: 12 days, mean duration: 26 days, responsible drugs: antibiotics: 41 p. 100, nonsteroidal antiinflammatory drugs: 11 p. 100, anticonvulsants: 10 p. 100. A few reactions showed a monomorphous pattern fitting a single denomination in a classic dermatological nomenclature: maculopapular: 10 p. 100, morbilliform: 6 p. 100, scarlatiniform: 5 p. 100, small papules: 5 p. 100. Polymorphous reactions were observed in 73 p. 100 of cases with 3 different patterns on the average. Nearly half of the cases exhibited maculopapular and scarlatiniform lesions simultaneously. We observed fever (61 p. 100), mucosal lesions (26 p. 100), lymphadenopathy (30 p. 100), eosinophilia (count > 500/mm3: 44 p. 100, > 1500/mm3: 17 p. 100), and visceral involvement (22 p. 100), including hepatitis (18 p. 100). Four severity markers were identified: fever, lymphadenopathy, erythema involving more than 60 p. 100 of the body surface area, eosinophilia. CONCLUSION: This study underlines the polymorphous clinical presentation of skin reactions to drugs and the minority of maculopapular or morbilliform patterns leading us to propose the term of "erythematous drug reactions". Despite several biases (hospital recruitment of more severe reactions, retrospective analysis) this study is the first to show some severity markers. Close to the criteria used to define the "hypersensitivity syndrome" these simple clinical markers may have important practical implications.

Age Factors↗
PubMed1999

[Hand-foot-mouth syndrome recurring during common variable deficiency].

BACKGROUND: Common variable immunodeficiency is characterized by hypogammaglobulinemia and recurrent bacterial infections. More uncommonly, these patients develop chronic enterovirus infectious meningoencephalitis. Recurrent enterovirus skin infection has not been reported to date in subjects with common variable immunodeficiency. CASE REPORT: A 26-year-old man had suffered repeated episodes of otorhinolaryngological and pulmonary infections since childhood. He experienced three episodes of vesicular cutaneous eruption involving the palms of both hands, the plantar aspect of the feet and the buccal mucosa. The patient was hospitalized in March 1995 at the third episode. Temperature was 38 degrees C. He had maculopapulous and vesicular eruptions on the palm of the hands and plantar aspect of the feet with irritation in some areas. Petichial lesions were seen on the palate. PCR demonstrated viral DNA and cell cultures of a lesion biopsy were positive for enterovirus. Gammaglobulinemia was 4 g/l with low B cell count. DISCUSSION: Viral infections are uncommon in patients with common variable immunodeficiency as cellular immunity remains normal. Severe viral infections caused by enteroviruses have however been reported, generally associating chronic, and generally fatal, meningoencephalitis. Our case would be the first case of a recurrent hand, foot and mouth disease in such patients.

Adult↗
PubMed1998

Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis.

BACKGROUND: Toxic epidermal necrolysis (TEN) is associated with a 30% death rate. Tumour necrosis factor alpha (TNF-alpha) has been implicated in the pathogenesis of TEN. Thalidomide is a potent inhibitor of TNF-alpha action. We did a double-blind, randomised, placebo-controlled study of thalidomide in TEN. METHODS: The patients received a 5-day course of thalidomide 400 mg daily or placebo. The main endpoint was the progression of skin detachment after day 7. Secondary endpoints were the severity of the disease, evaluated with the simplified acute physiology score (SAPS), and the mortality. TNF-alpha and interleukin 6 were measured. FINDINGS: The study was stopped because there was excess mortality in the thalidomide group--ten of 12 patients died compared with three of ten in the placebo group (Fisher's exact test with Katz's approximation, relative risk=2.78, p=0.03). After adjustment for SAPS, mortality remained significantly higher in the thalidomide group than in the placebo group (exact logistic regression mid-p=0.007; 95% CI for odds ratio 2.7 to infinity). Plasma TNF-alpha concentration was higher in the thalidomide group than the placebo group on day 2, though the difference was not significant (Wilcoxon rank-sum test p=0.07). INTERPRETATION: Even though few patients were included, our data suggest that thalidomide is detrimental in TEN, possibly because of a paradoxical enhancement of TNF-alpha production.

Adult↗
PubMed1998

Vulvovaginal involvement in toxic epidermal necrolysis: a retrospective study of 40 cases.

OBJECTIVE: To determine the incidence, features, and surgical treatment of vulvovaginal lesions in toxic epidermal necrolysis. METHODS: Acute genital lesions were studied retrospectively in 40 women hospitalized for toxic epidermal necrolysis in a dermatologic intensive care unit. A questionnaire was sent to evaluate sequelae and their effects on sexual activity. Examination and surgical treatment were proposed to patients with symptomatic sequelae. RESULTS: Twenty-eight of the 40 patients reported genital lesions during the acute phase of toxic epidermal necrolysis. No specific treatment was carried out during the acute period. Sequelae were observed in five cases, of which three involved the lower genital tract and two the vulva exclusively. The two patients with exclusive vulval involvement did not attempt any sexual activity. The other three patients with both vulval and vaginal lesions were unable to have normal sexual intercourse. Two of the three patients were treated surgically. One patient succeeded in having intercourse, but surgery failed to relieve dyspareunia. CONCLUSION: Genital involvement is frequent during toxic epidermal necrolysis but rarely leads to symptomatic sequelae. Surgery for synechiae is sometimes necessary to recover sexual activity because the vulvovaginal canal is stenotic. Because of the partial effect on pain relief after surgery, a preventive approach should be tried.

Adolescent↗
PubMed1998

Lichenoid erythrodermic bullous pemphigoid of the African patient.

BACKGROUND: Lichen planus (LP) pemphigoides is defined by the concurrence of bullous pemphigoid (BP) and LP lesions. However, other autoimmune bullous skin diseases such as paraneoplastic pemphigus or bullous lupus erythematosus may also be associated with lichenoid eruptions. OBJECTIVE: Our purpose was to describe 3 African patients with a similar form of lichenoid erythrodermic BP. METHODS: Medical records of the 3 patients were reviewed for clinical, histologic, and biologic data. The ultrastructural localization of autoantibody deposits was studied by direct and indirect immunoelectron microscopy. The antigenic specificities recognized by the patients' sera were analyzed by immunoblotting on human epidermal extracts. RESULTS: Three black African men had a severe lichenoid erythroderma associated with BP lesions involving the skin and mucosa. Histologic and direct immunofluorescence examination of skin and mucosal lesions were consistent with the diagnosis of BP associated with a lichenoid dermatitis. Immunoblot analysis of sera detected anti-BPAG1 and anti-BPAG2 antibodies and immunoelectron microscopy showed IgG deposits localized in the lamina lucida and the hemidesmosomes. The 3 patients were natives of an area near the Senegal River and had the common HLA-DR10 haplotype. CONCLUSION: The clinical and histologic features of these 3 patients were distinct from classic LP pemphigoides. Their common geographic origin and the presence of the HLA-DR10 haplotype suggest that these cases represent a particular type of lichenoid erythrodermic BP, which is probably determined by genetic factors.

Adult↗
PubMed1998

The effect of addition of calcipotriol ointment (50 micrograms/g) to acitretin therapy in psoriasis.

Our purpose was to find out whether the addition of calcipotriol ointment (50 micrograms/g) to systemic treatment with acitretin produces additional therapeutic effects and thereby an acitretin-sparing effect, and further to investigate the safety and tolerability of this combination. A multicentre, randomized, double-blind placebo-controlled study was designed. Patients were randomized to receive calcipotriol or placebo. All patients were treated with a starting dose of 20 mg acitretin per day and doses were adjusted at 2-weekly intervals with increments of 10 mg per day up to a maximum of 70 mg per day. The dose requirement for acitretin, clinical signs and adverse events were recorded. Seventy-six patients were randomized to treatment with calcipotriol 50 micrograms/g ointment twice daily and 59 patients to treatment with the vehicle only twice daily. Clearance or marked improvement was achieved by 67% of the patients in the calcipotriol group and by 41% of the patients in the placebo group (P = 0.006). Calcipotriol treatment proved to have a statistically significant additional effect to acitretin on the Psoriasis Area and Severity Index, redness, thickness and scaliness as compared with placebo. Clearance or marked improvement was achieved with a statistically significantly lower cumulative dose of acitretin by the patients in the calcipotriol group as compared with the placebo group. The number of patients reporting adverse events was pronounced and largely related to acitretin. No significant differences were observed between the two treatment groups with respect to adverse events. Laboratory assessments were essentially normal. The addition of calcipotriol ointment to acitretin treatment contributes to the efficacy, reduces the cumulative dose of acitretin to reach marked improvement or clearance, and is well-tolerated and safe.

Acitretin↗