Search PubMed⌕ Search

Biomedical subjects

C Bedane

Publications and source records attributed to C Bedane.

At least 37 records · Page 2Linked to original sources
PubMed2000

[Pretibial epidermolysis bullosa and hypothyroidism].

BACKGROUND: We report a case of primary non-autoimmune hypothyroidism causing pretibial epidermolysis bullosa. CASE REPORT: A 70-year-old man with primary non-autoimmune hypothyroidism developed blisters of different ages on the lateral aspect of both legs. Pathology reported blisters with subepidermal cleavage. Direct immunofluorescence was negative. Electron microscope examination showed a variable cleavage level and diffuse infiltration of a granulous and amorphous microfibrillar substance. After hormone replacement therapy, euthyroidism was associated with a reduction in the number of bullae and finally complete remission. After 12 months follow-up, the patient has not experienced recurrence. DISCUSSION: Recurrence-free clinical improvement after hormone replacement therapy suggests the diagnosis of hypothyroidism pretibial epidermolysis bullosae. Mochizuki et al. described a similar case which rapidly regressed after hormone therapy but where the electron microscope showed a different cleavage level. These bullae appear to result from a mechanical mechanism due to their localization in areas exposed to friction and also to the presence of bullae of different ages. This hypothesis is confirmed by the presence of a variable level of cleavage and a substance dense to electrons at electron microscopy as well as by the skin weakness. Our case confirms the reality of hypothyroidism pretibial epidermolysis bullosa. Thyroid hormones should be assayed in patients presenting pretibial bullae.

Aged↗
PubMed2000

[Scurvy presenting with ecchymotic purpura and hemorrhagic ulcers of the lower limbs].

INTRODUCTION: The risk of vitamin C deficiency is underestimated in industrialized countries and is only disclosed in rare cases of severe scurvy. CASE REPORT: We report three cases of scurvy presenting with ecchymotic purpura and hemorrhagic ulcerations of the lower limbs. Vitamin C supplementation led to rapid improvement of the skin lesions. DISCUSSION: Clinical diagnosis of low-grade deficiency can be difficult. Biological diagnosis requires special care in sample taking and transport.

Aged↗
PubMed1999

Toxicity and feasibility of adjuvant high-dose interferon alpha-2b in patients with melanoma in clinical oncologic practice.

To assess the feasibility and toxicity profile of high-dose interferon alpha-2b (IFN-alpha-2b) in the adjuvant treatment of patients with cutaneous malignant melanoma outside the reference ECOG 1684 clinical trial, we conducted a prospective follow-up in an identical population of patients (cutaneous melanoma, T4 and/or N1) treated by intravenous IFN-alpha-2b:20 MIU m(-2), 5 days a week for 4 weeks; and subcutaneous:10 MIU m(-2), 3 times a week for 11 months. Thirty-six consecutive patients were considered in four different institutions. The frequency and severity of side-effects related to IFN-alpha, as well as the percentage of the planned dose given to patients, were identical to those reported in the initial report by ECOG. Fifty per cent and 47% of patients had a grade 3/4 WHO toxicity in the induction and consolidation phase respectively. A dose modification was necessary for 47.2% and 55.8% of the patients in the induction and consolidation phase respectively. The schedule and dose of high-dose IFN-alpha-2b in the adjuvant treatment of cutaneous malignant melanoma, as reported by ECOG 1684, is feasible. The significant toxicity reported in ECOG 1684 was also seen in our patients. Nevertheless, this protocol will not be a 'standard' treatment until the publication of the ECOG 1690 trial.

Antineoplastic Agents↗
PubMed1999

[Penile lichen sclerosus: follow-up study].

INTRODUCTION: The aim of this work was to study the long-term course of patients with penile lichen sclerosus and atrophicus. PATIENTS AND METHODS: We reviewed the files of 16 patients followed at our university center from 1982 to 1997 for pathologically proven penile lichen sclerosus and atrophicus. Age at disease onset, duration of the disease course, presence of a triggering factor, initial localization, signs and symptoms, treatment given and its efficacy were recorded. RESULTS: Mean age at diagnosis was 52 years. Inaugural prepuce localizations were observed in 6 cases, involving the distal part of the penis and the glan in 3 cases. Concomitant involvement of the glan and the balanopreputial groove were seen in 6 cases. The localization was limited to the glan in one case. Six patients underwent posthectomy and one had dorsal plasty of the prepuce. The 9 other patients were treated medically with topical corticosteroids and/or androgens. One patient had died prior to evaluation. Among the 9 patients given medical treatment, lichen sclerosus persisted in 7, one developed a squamous cell carcinoma of the penis and one was cured. Among the 6 cases of surgical treatment, 5 were cured and 1 had persistent lesions. Considering the initial localization, long-term course showed that 7 of the 8 patients with a prepuce localization (alone or in association with glan involvement) were cured while the 7 patients with a lesion of the balanopreputial groove (alone or in association with glan involvement) or a lesion of the glan alone still had lichen sclerosus and atrophicus. The non parametric Fischer test showed that the chances of cure were better for preputial localizations than for balanopreputial localizations (p = 0.001) and that surgical treatment was more effective than medical treatment. DISCUSSION: This study of a small number of patients highlighted two points: prepuce localizations of lichen sclerosus and atrophicus cure better than balanopreputial localizations, and that medical treatment is partially effective in balanopreputial localizations. One patient developed squamous cell carcinoma of the penis. As the level of the cancer risk remains unknown, it would be important to provide the most effective treatment in all cases of penis lichen sclerosus and atrophicus.

Adult↗
PubMed1998

Evaluation of clinical criteria for diagnosis of bullous pemphigoid. French Bullous Study Group.

OBJECTIVE: To check the potential usefulness of clinical criteria for the diagnosis of bullous pemphigoid when state-of-the-art techniques such as Western immunoblotting, immunoprecipitation, and indirect immunofluorescence on salt-split skin or direct immunoelectron microscopy are not available. DESIGN: Comparison of the clinical criteria between 2 groups (with and without bullous pemphigoid) as defined by immunoelectron microscopy used as standard criterion, in a prospective study. Multivariate logistic regression analysis was carried out by including all items that were statistically significant (at P < .05 level) in univariate analysis. SETTING: Five dermatology departments in teaching hospitals. PATIENTS: The 231 patients studied had subepidermal autoimmune bullous diseases with linear IgG or C3 deposits in the basement membrane zone (157 with bullous pemphigoid, 33 with cicatricial pemphigoid, 30 with epidermolysis bullous acquisita, 5 with lupus erythematosus, and 6 others). A second set of patients was used to calculate predictive values. RESULTS: The multivariate logistic stepwise analysis resulted in a final set of predictors that included only 4 items: absence of atrophic scars, absence of head and neck involvement, absence of mucosal involvement, and age greater than 70 years. No additional variables met the .05 significance level to enter into the model. If 3 of these 4 characteristics were present, a diagnosis of bullous pemphigoid could be made with a sensitivity of 90% and a specificity of 83%; these predictive values were calculated on a sample of 70 new cases. CONCLUSIONS: With and estimated incidence of bullous pemphigoid among subepidermal autoimmune bullous diseases of 80%, the presence of 3 of the 4 significant criteria allows the diagnosis of bullous pemphigoid, with a positive predictive value of 95%. Our set of clinical criteria thus allows the diagnosis of bullous pemphigoid with good validity for both clinical practice and therapeutic trials.

Aged↗
PubMed1998

Acquired junctional epidermolysis bullosa associated with IgG autoantibodies to the beta subunit of laminin-5.

We report the case of a 72-year-old man with clinical features resembling those of non-lethal junctional epidermolysis bullosa associated with IgG autoantibodies to the beta chain of laminin-5. The patient presented with a sudden onset of blistering and severe fragility of the skin and mucous membranes resulting in atrophic scars. Electron microscopy showed that the blistering arose in the lamina lucida. Indirect immunofluorescence indicated that the autoantibodies bound to the dermal side of 1 mol/L NaCl-split skin, and both direct and indirect immunoelectron microscopy demonstrated antibody binding to the lamina densa. Postembedding immunogold electron microscopy also revealed labelling in the lamina lucida beneath the hemidesmosomes. On immunoblotting, we found the autoantibodies to comigrate with the beta chain of laminin-5. Following the nomenclature of inherited junctional epidermolysis bullosa with mutations of the laminin-5 gene, we propose the name acquired junctional epidermolysis bullosa for this newly recognized disease.

Aged↗
PubMed1998

Pyoderma gangrenosum complicating Cogan's syndrome.

Cogan's syndrome is a rare clinical entity defined by the association of a nonsyphilitic interstitial keratitis and vestibuloauditory dysfunction, typically Menière's disease-like; the condition has been reported in association with a variety of cutaneous diseases. We now report a case of pyoderma gangrenosum complicating Cogan's syndrome in a 57-year-old woman, which then healed dramatically, as more interestingly did the associated uveitis with minocycline therapy.

Ataxia↗
PubMed1997

Postoperative interstitial radiotherapy of keloids by iridium 192: a retrospective study of 46 treated scars.

BACKGROUND: Keloids are clinically vexacious scars characterized by a high recurrence rate after excision alone (50-100%). Many adjuvant techniques have been used with more or less convincing results. OBJECTIVE: The aim of this study is to show the efficiency of keloidectomy and postoperative interstitial radiotherapy by an iridium 192 wire. METHODS: During a 14-year period, 39 patients with keloids (46 keloids) were treated by this regimen. Seven patients had been previously treated by intralesional corticosteroids, surgery alone or postoperative interstitial radiotherapy, without improvement. A dose of 12 Gy (7 keloids) to 15 Gy (22 keloids) was delivered at a point 2.5 mm from the axis of the wire. For 17 keloids the dose schedule was 15 Gy at a point 5 mm to the wire. The median follow-up was 7 months. RESULTS: The overall success rate is close to 63%. A recurrence occurred in 14 cases without relation to the method used, the age or the localization of the lesions. There were no side effects observed. CONCLUSIONS: Postoperative interstitial radiotherapy represents an effective, unconstraining and safe treatment for keloids if the contraindications are respected.

Adolescent↗
PubMed1997

Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients.

The prognosis of bullous pemphigoid (BP), a disorder which usually affects elderly patients, is not well established and conflicting data have been reported about the mortality rate of the disease. Our objective in this study was to assess the clinical and immunological factors determining survival in a prospective series of 94 patients with BP. A cohort of 94 consecutive patients with BP (mean age +/- SD: 81 +/- 4 years) was studied over an 8-year period (1987-94) in one department and patients followed up for at least 1 year. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (i.e. linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting. Our analysis (median duration of follow-up: 5 years) showed that 37% of BP patients were dead within a year of starting treatment. The clinical or immunological factors which may influence the prognosis of BP were studied according to the criterion of death or survival by the end of the first year of treatment. None of the following factors was found to be significantly linked to the prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, or the presence of circulating basement membrane zone autoantibodies by indirect IF. An impaired general condition and a history of coronary artery disease indicated a bad prognosis. The presence of circulating autoantibodies against BP180 autoantigen but not autoantibodies against BP230, as detected by immunoblotting on epidermal extracts, was found to be significantly more frequent (60% vs. 25%) in BP patients who died within the first year of treatment (P < 0.01). We conclude that the presence of circulating autoantibodies against BP180 represents the first intrinsic prognostic factor that has been demonstrated in BP. This result supports the growing body of evidence for the pathophysiological importance of the anti-BP180 autoantibodies.

Aged↗
PubMed1997

[Pemphigoid vegetans. An immunoelectron microscopic study].

INTRODUCTION: Pemphigoid vegetans is a rare disease. It has a clinical resemblance to pemphigus vegetans, but there are histological and immunopathological features of bullous pemphigoid. CASE REPORT: We describe a case in a 57-year-old-man who had developed intertriginous vegetating plaques. Histologic examination of a skin biopsy specimen and direct immunofluorescence microscopy of a biopsy specimen were those of bullous pemphigoid. Immunoblot studies and indirect immunofluorescences of salt-split skin were negative. Direct immunoelectron microscopy was consistent with bullous pemphigoïd. DISCUSSION: Only five cases have been reported. We describe the first case including direct immunomicroscopic findings which suggest that pemphigoid vegetans is a subtype of bullous pemphigoid. The other interest was a remarkable improvement with tetracyclines.

Anti-Bacterial Agents↗
PubMed1997

[Waldenstrom's macroglobulinemia with antibasement membrane activity of monoclonal immunoglobulin].

INTRODUCTION: Waldenström macroglobulinemia is a rare hematologic disorder with characteristic malignant plasma cell proliferation associated with the secretion of a IgM monoclonal immunoglobulin which is the cause of most of the clinical manifestations. Skin involvement is exceptional. We report a new case of Waldenström macroglobulinemia discovered in a patient who developed specific skin lesions with monoclonal immunoglobulin deposits. CASE REPORT: A 50-year-old woman developed spontaneously painful infiltrated erythematopapular plaques over the extension aspects of the limbs. There were no other clinical manifestations. The patient had a kappa IgM monoclonal gammapathy. The diagnosis of Waldenström macroglobulinemia was proposed after the discovery of voluminous intra-abdominal adenopathies and was confirmed by immunohistochemistry of the biopsies, indirect immunofluorescence evidenced reactivity against the epidermal basal membrane. Chemotherapy led to successful regression of the skin lesions, the tumoral mass and circulating IgM. DISCUSSION: This is the second reported case of Waldenström macroglobulinemia with anti-basal membrane monoclonal immunoglobulin. This IgM would recognize an 82 kD antigen on the dermal side of the dermo-epidermal junction situated in the anchoring fibres of the lamina densa, a zone classically involved in acquired bullous epidermolysis.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1996

Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain.

Cicatricial pemphigoid (CP) is an autoimmune blistering disease that primarily affects mucosal tissues. Autoantibodies to laminin-5 have previously been detected in certain patients with a CP-like disease; however, individuals that exhibit this reactivity profile apparently represent a small subset of CP patients. In the present investigation, 0 of 18 CP sera showed reactivity with laminin-5 by immunoblotting. In contrast, 18 of 23 CP sera (78%) recognized a 180-kDa epidermal antigen that, by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, co-migrated with BP180, a hemidesmosomal glycoprotein associated with two other autoimmune blistering diseases, bullous pemphigoid and herpes gestationis. To investigate further the CP autoimmune response, various segments of human BP180 were expressed as bacterial fusion proteins and assayed by immunoblotting for reactivity with CP patients' sera. The results of this investigation demonstrated that the BP180 autoantigen is indeed a major target of CP autoantibodies. Further, two distinct CP-reactive sites were identified on the extracellular domain of the BP180 protein, one located in the non-collagenous (NC) 16A domain (at or near the previously defined autoantibody-reactive site recognized by bullous pemphigoid and herpes gestationis sera) and the other in the carboxy-terminal region of this protein. Sixteen of 23 CP sera (70%) reacted with one or both of these antigenic sites of BP180. Other immunologic data suggested that BP180 may harbor additional CP-reactive sites. In conclusion, there are now three bullous diseases, bullous pemphigoid, herpes gestationis, and cicatricial pemphigoid, that are known to be associated with an autoimmune response against the extracellular domain of the BP180 antigen.

Aged↗