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Biomedical subjects

C Bedane

Publications and source records attributed to C Bedane.

At least 55 records · Page 3Linked to original sources

Thalidomide and recurrent aphthous stomatitis: a follow-up study.

BACKGROUND: Thalidomide is used for the symptomatic treatment of recurrent aphthous stomatitis (RAS). Some authors reported remissions, but this was not evaluated. OBJECTIVE: To evaluate the number of patients who could stop or reduce thalidomide treatment. METHODS: Twenty-five patients with RAS treated with thalidomide and followed during at least 1 year were retrospectively studied. RESULTS: Six patients could stop the treatment and further presented minor aphthae, 10 needed minimal daily doses of thalidomide and 7 did not respond to 100 mg daily. One patient was not evaluated because of an early side effect and one was lost to follow-up. CONCLUSION: This study showed that a minority of patients responded and could stop thalidomide therapy whereas another group of patients could be maintained in remission with low doses of thalidomide which may represent a means to reduce the potentially severe side effects.

Adult↗

[Prenatal diagnosis of hereditary bullous epidermolysis. A case report].

A premature infant born to a consanguinous couple (mother's age = 27 years) presented Hallopeau-Siemens disease and died at 3 weeks. At a second pregnancy, fetal skin biopsies at 21 weeks gestation demonstrated the absence of the disease. The fetus died in utero at 31 weeks of an unknown cause. A third pregnancy was carried to term successfully and terminated by delivery of a normal infant. Unlike most hereditary bullous epidermolyses, the severe prognosis of Hallopeau-Siemens disease justifies antenatal diagnosis as does Herlitz disease, another familial disease. Fetal skin biopsy at 21 weeks is classically performed, but localization of the genetic abnormalities would suggest that a simple trophoblast biopsy during the first trimester may be sufficient.

Adult↗

[Neuroendocrine primary cutaneous carcinoma. Therapeutic aspects in 13 patients].

INTRODUCTION: Since the description by Toker in 1972, neuroendocrine carcinoma or Merkel cell carcinoma, is a well identified clinical entity although the appropriate treatment is still debated. Wide surgical exeresis is indicated as first line treatment in all cases but the question concerning protocols for adjuvant radiotherapy or chemotherapy remains open. We analyzed retrospectively our series of 13 patients with neuroendocrine carcinoma looking for an association between radiotherapy and surgery. PATIENTS: There were 7 women and 6 men in our study population (age range 43-88 years). Two cancers of the rectum and one prostate cancer were associated. In 6 cases, the tumor was localized on the limbs, in 5 on the face and in 2 on the buttocks. Mean delay to diagnosis was 2.5 months. At diagnosis, only 1 patient had satellite nodes. The pathology examination evidenced intermediary cell type and architecture. Immunohistochemistry tests were positive for NSE, NF, KII and the ultrastructure confirmed the diagnosis. Nine patients were treated with surgical exeresis with wide 1 cm margins and was completed with radiotherapy of the tumoral bed at homogeneous doses of 45 to 60 Gy. Surgery alone was used in 4 patients. RESULTS: Mean follow-up was 27 months (5-98). Among the 13 operated patients, 11 have survived, 1 died due to the neoplasia and in 1 other from another cause. Local and regional recurrence rate after exercise alone was 50 p. 100 (2/4) with concomitant development of metastasis in both cases. When local or locoregional post-operative radiotherapy was given, local or regional recurrence rate was 33 p. 100 (2/6). DISCUSSION: Cutaneous neuroendocrine carcinoma or Merkel cell carcinoma is an uncommon skin tumor. It is difficult to determine the management protocol on the basis of data in the literature. Due to risk of locoregional recurrence, we currently propose post-operative radiotherapy of the tumor bed and drainage nodes in all patients.

Adult↗

[Multicentric histiocytosis with hematological involvement].

INTRODUCTION: The aim of this work was to present a case of multicentric histiocytosis with haematologic involvement. CASE REPORT: A 68-year-old man presented with poor general health and a nodular eruption of the skin and larynx. On clinical examination there was an enlarged spleen and laboratory results revealed an inflammatory syndrome, platelet count 60,000 and myelemia with 10 p. 100 immature elements in a white cell count of 14,000. Pathology and ultrastructure examinations led to the diagnosis of multicentric histiocytosis. Bone marrow aspiration was normal. Pancytopenia then developed with bone marrow hypoplasia without infiltration. Corticosteroids then cyclophosphamide were uneffective for either the skin disease or the hematologic disorder. The patient developed severe buccal aphthosis which responded well to thalidomide. This treatment reduced the size and the number of skin nodules. Finally, renal failure of unknown origin was observed. DISCUSSION: Electron microscopy is essential for positive and differential diagnosis of atypical multicentric histiocytosis. Hematological disorders associated with multicentric histiocytosis may either be specific or totally independent.

Aged↗

Treatment of verrucous carcinoma with recombinant alfa-interferon.

BACKGROUND: Verrucous carcinoma is an uncommon neoplasm whose medical treatment is not well defined. OBJECTIVE: We report the clinical effects of recombinant alpha-interferon (IFN) with varying dispensing routes on the tumor size in 3 patients exhibiting three different subtypes of verrucous carcinoma. OBSERVATIONS: In case 1, an oral florid papillomatosis of the upper gingiva, a stabilization of the lesion size was observed after the administration of a cumulative dose of 45 million IU of IFN alfa-2b. The patient died after discontinuation of the treatment due to hepatic toxicity. In case 2, a giant condyloma acuminatum of the penis, treatment with a cumulative dose of 522 million IU of IFN alfa-2a first resulted in stabilization of the disease, and no evidence of recurrence was observed after surgical treatment. In case 3, a verrucous carcinoma of the leg, a mild decrease in lesion size was observed after the administration of a cumulative dose of 174 million IU. Surgery was finally required. CONCLUSION: In reducing the growth of the tumor, treatment by IFN appears to be an adjuvant therapy of verrucous carcinoma, but it never prevents surgery or death.

Aged↗

Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group.

BACKGROUND AND DESIGN: The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 months in three university dermatologic centers in Amiens, Limoges, and Tours, France, that correspond to a cumulative reference population of 3.55 x 10(6). RESULTS: Using data from these regions, we found a mean annual incidence of autoimmune subepidermal bullous diseases to be 10.4 per million people and, therefore, estimated the overall number of new cases of these disorders in France to be about 590 cases per year. According to clinical and immunoelectron microscopic criteria, a precise diagnosis was established in 94 cases, distributed as follows: bullous pemphigoid, 69 cases; cicatricial pemphigoid, 12 cases; linear IgA dermatosis, five cases; herpes gestationis, four cases; epidermolysis bullosa acquisita, two cases; and vesiculobullous systemic lupus erythematosus, two cases. CONCLUSION: Our prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data. Our estimated incidence of bullous pemphigoid (seven new cases per million people per year) is large enough to establish bullous pemphigoid as the major autoimmune subepidermal bullous disease for the purpose of therapeutic trials. On the contrary, all other disorders, particularly epidermolysis bullosa acquisita (estimated annual incidence, 0.17 to 0.26 per million people), were very rare and reflect the paucity of patients available for short-term clinical studies in France.

Adult↗

[Ultrastructural localisation of pemphigus vulgaris and pemphigus foliaceus antigens by indirect immunoelectron microscopy. Apropos of 7 cases].

INTRODUCTION: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases characterized by loss of cell-cell adhesion and by autoantibodies directed against epidermal cadherins. The ultrastructural localization of PV antigen remains controversial, whereas the location of PF antigen seems to be established. The use of different techniques could explain these various data. To investigate this matter, indirect immunoelectron microscopy (IEM) and Western blot analysis on bovine tongue epithelium were used. MATERIAL AND METHOD: Serum samples from patients with PF(3), PV (4) and control samples from healthy patients (2) were analysed in this study. The inclusion criteria were based upon characteristic clinical features, level of epidermal cleavage on histological preparations and presence of circulating anti-epithelial cell surface antibodies. Indirect IME was performed on normal human skin. Peroxidase labelling was used. Serum samples were also analysed by western immunoblotting on bovine tongue epithelium. RESULTS: Indirect IEM examination of PV sera showed immune deposits located both on desmosomal and extra-desmosomal areas, whereas in PF, IgG deposits were strictly localized on desmosomal structures. By Western blot analysis, PV sera recognized a 130 kDa polypeptide and PF sera a 150 kDa polypeptide. DISCUSSION: Indirect IEM on normal human skin using peroxidase labelling was used because of the best antigenic conservation obtained. Our results suggest that PV antigen could exist both on desmosomal junctions and adherens junctions, whereas PF antigen (desmoglein I) is restricted to desmosome.

Adult↗

[Bacterial dermo-hypodermatitis in adults. Incidence and role of streptococcal etiology].

INTRODUCTION: The frequency of bacterial dermohypodermal infections would appear to be increasing in western countries, particularly severe necrotizing forms. PATIENTS AND METHODS: We attempted to determine: (1) the incidence of the two most well-defined forms of bacterial dermohypodermal infections (erysipelas and necrotizing fasciitis) in a retrospective study of hospitalized cases seen over a period of 13 years in the Limoges University Hospital and (2) the role of streptococci as causative agents in bacterial dermohypodermal infections in the adult by collecting bacteriological data in 174 cases of erysipelas and 31 cases of necrotizing fasciitis. RESULTS: This retrospective analysis confirmed that the annual incidence of erysipelas was constantly increasing over the study period (1978-1991) while the annual incidence of necrotizing fasciitis was much lower and more constant. The two incidence curves were not parallel. Bacteriology, detection of streptococci (groups A, B, C or G) in skin specimen was better with direct immunofluorescence (64 p. 100) than with latex agglutination (47 p. 100) or classic culture techniques (28 p. 100) for erysipelas. Streptococci were determined to be the causative agent in 79 p. 100 (137/174) of the cases of erysipelas using at least one of the 3 bacteriological tests and/or serology. Group A streptococci predominated (67 p. 100 of the cases). For necrotizing fasciitis, streptococci was identified with classical culture technique, latex agglutination and/or serology in 12/131 cases (39 p. 100), again with a group A predominance (7 cases). In the other cases, multiple microbial flora (12 cases) were found including Staphylococcus aureus (3 cases) and Serratia liquefaciens (1 case). CONCLUSION: These results show that streptococci (group A predominantly and group G to a lesser extent) were the major, though not exclusive, causative agents in bacterial dermohypodermal infections in the adult, including necrotizing forms.

Adult↗

[Inguinal mastocytosis with histiocytic infiltrate].

INTRODUCTION: The diagnosis of mastocytosis is usually not difficult in cases with typical skin lesions. Intertrigo is exceptional. CASE REPORT: An 83-year-old patient developed inguinal intertrigo bilaterally. The lesions contained maculopapulous elements without Darier's sign. The histology examination revealed a mixed mast cell infiltration predominated by histiocytes. The ultrastructure confirmed the diagnosis of cutaneous mastocytosis. No extension was observed. DISCUSSION: Skin folds are rarely involved in infiltrating diffuse mastocytosis. Two cases have been reported in the literature including one doubtful case. Mastocytosis should thus be included in the possible diagnoses in patients with macupopapulous intertrigo. In addition, the mastocytic and histiocytic polymorphism observed here is rare.

Aged↗

[Survival prognosis in pemphigoid. A cohort analysis of 78 patients].

INTRODUCTION: Bullous pemphigoid (BP) is the most frequent autoimmune bullous skin disease and usually affects elderly patients. Despite some conflicting data, vital prognosis is poor in BP, of which mortality rate after one year of treatment varies between 10 and 40 p. 100. To date, the factors influencing this prognosis remain to be determined. PATIENTS AND METHODS: A cohort of 78 consecutive patients with BP (mean age: 80 +- 4 years) has been prospectively selected on a six-year period (1987-1992) in Limoges. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting on epidermal extracts. RESULTS: The follow-up analysis (mean duration of follow-up: year of treatment (22 p. 100 in the first three months) with an actuarial survival curve inclined to be horizontal by the end of this first year of treatment. Therefore, we have studied the clinical or immunologic factors susceptible to influence the vital prognosis of BP according to this main criterion: death (or survival) at the end of the first year of treatment. None of the following factors has been found to be significantly linked to the vital prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, presence of circulating basement membrane zone autoantibodies by indirect IF. The clinical factors of bad prognosis were an altered general condition and a history of coronaropathy. The presence of circulating autoantibodies against BP180 antigen (but not the one of autoantibodies against BP230) as detected by immunoblotting on epidermal extracts was found to be significantly more frequent (50 vs 22 percent) in BP patients who died within the first year of treatment (p < 0.02). DISCUSSION: Although modestly discriminating, the presence of circulating autoantibodies against BP180 represents the first vital prognosis factor demonstrated in BP. This result confirms the growing pathophysiologic importance of the anti-BP180 autoantibodies of which the pathogenic role has been recently formally demonstrated in BP using an animal model.

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[Cutaneous reactions to gold salts].

OBJECTIVE: Evaluate the clinical features of skin reactions to gold salts. INTRODUCTION: Gold dermatitis is described in the literature as a group of reactions non-specifically associated with these drugs. PATIENTS AND METHODS: Ten patients (7 males, 3 females, mean age 59.6 years [corrected] with a skin reaction were studied over a 6 year period. All were treated with Allochrysine for rheumatoid arthritis (n = 8), rhizomelic pseudopolyarthritis (n = 1) or arthropathic psoriasis (n = 1). RESULTS: The delay to the first signs was from 2 weeks to 8 months. Three lichenoid eruptions (including two with buccal lesions and two which followed an autonomous course), 2 pityriasis rosea (one with eosinophilia and one with liver disease), 2 eczematoid dermatoses and one urticaria were observed. Pathology examinations (8/10) were in agreement with clinical diagnosis. Imputability was 14 (1 case), 13 (8 cases) and 12 (1 case). DISCUSSION: The clinical features observed were variable and in agreement with a particular clinical situation. They were not specific to gold salts and cannot be qualified as "gold dermatitis". Two autonomous and severe lichenoid eruptions were observed. Generalized lesions were associated with biological signs. This situation must be considered as a marker of severity.

Adult↗

[Multocida Pasteurella and leg ulcer].

INTRODUCTION: We observed two cases of Pasteurella and discuss the role of bacterial sampling in ulcerations of the lower limb. CASE REPORTS: In the first case, Pasteurella was discovered in an ulceration of the lower limb and was cured with no particularly specific care. In the second case, Pasteurella had been inoculated by scratching an ulceration and could not be cured without specific treatment. DISCUSSION: No specific pathological consequence in chronic carriers could be possible, a situation which has often been reported in the literature. Pasteurella is an unusual specific cause of ulcerations of the lower limb.

Aged↗

[Disulone hypersensitivity syndrome].

Hypersensitivity to dapsone (Disulone) is a rare side effect of sulfone therapy. The diagnosis is based on variable clinical manifestations and laboratory findings dominated by hyperthermia, skin eruptions and a mixed form of hepatitis. We observed a case of hypersensitivity to dapsone in a 57-year-old woman who was treated with Disulone for cicatricaial pemphigoid. Clinical manifestations and laboratory results returned to normal 3 weeks after drug withdrawal. This case is interesting because the skin eruptions classically described were not observed. In addition this is apparently the first case reported of such a hypersensitivity syndrome occurring during treatment of autoimmune bullous dermatosis.

Chemical and Drug Induced Liver Injury↗

[Ehlers-Danlos type IV syndrome presenting as renovascular hypertension].

The association of Ehlers-Danlos type IV syndrome and acrogeria with renovascular hypertension is reported. Ultrastructural abnormalities observed in our case were different of those associated with acrogeria. We propose that acrogeria associated with Ehlers-Danlos type IV syndrome be a different disease of acrogeria of Gottron. The occurrence of renovascular hypertension in Ehlers-Danlos is unusual.

Adult↗