Biomedical subjects

Manuelle Viguier

Publications and source records attributed to Manuelle Viguier.

7 recordsLinked to original sources

PubMed2004

Treatment of refractory pemphigus vulgaris with rituximab (anti-CD20 monoclonal antibody).

BACKGROUND: Pemphigus vulgaris (PV) is a severe antibody-mediated autoimmune blistering disease. Because some patients with PV do not enter into remission, despite the use of high-dose corticosteroid therapy and immunosuppressive adjuvant treatments, new effective and safer agents are warranted to treat refractory PV. Rituximab, a monoclonal anti-CD20 antibody, induces depletion of B cells in vivo and has shown efficacy in patients with refractory antibody-mediated autoimmune disorders. We describe herein 3 patients treated with rituximab for severe PV. OBSERVATIONS: Three patients with refractory PV were treated with rituximab, resulting in a clinical response in all patients, which was complete in 2 patients. A decline in titers of circulating antiepidermis autoantibodies paralleled disease activity, while circulating B cells remained undetectable for 6 to 10 months. Two patients experienced bacterial infection in the weeks following the rituximab course. A clinical relapse occurred in 2 patients, at 6 and 10 months. A second course of rituximab controlled the disease in one of them. CONCLUSION: These patients' response suggests that rituximab may be a valuable treatment for refractory PV and warrants further studies to evaluate the risk-benefit ratio in patients with PV showing resistance to classic therapy.

Adult↗

PubMed2003

[Psoriasis].

Explore the source record for details and available documents.

Diagnosis, Differential↗

PubMed2003

Arsenic trioxide induces apoptosis of cutaneous T cell lymphoma cells: evidence for a partially caspase-independent pathway and potentiation by ascorbic acid (vitamin C).

Arsenic trioxide (As2O3) displays apoptogenic properties against various types of hematopoietic malignancies. We investigated the effects of As2O3 on the viability of the cutaneous T cell lymphoma cell lines HuT-78, SeAx, and Myla, and of peripheral blood mononuclear cells from patients with Sézary syndrome, by using propidium iodide and annexin-V staining, terminal deoxyuridine triphosphate nick end labeling (TUNEL), cell cycle analysis, mitochondrial transmembrane potential (delta psi(m)) alterations, cytochrome c release, and detection of processed caspase-3. We also report in vivo effects of As2O3 in two patients with cutaneous T cell lymphoma. The results show that As2O3 induces apoptosis of cutaneous T cell lymphoma lines and of Sézary cells from patients in a time- and concentration-dependent manner in vitro, as demonstrated by annexin-V staining, mitochondrial depolarization, and DNA fragmentation. Ascorbic acid 100 microM potentiated As2O3-induced Sézary cell death, whereas interferon-alpha had no synergistic effect. As2O3-induced Sézary cell death involves activation of caspase-3, cleavage of poly(ADP-ribose)polymerase, and cytochrome c release, but was only partially inhibited by the pancaspase inhibitor Z-VAD.fluoromethylketone. Finally, As2O3 was administered to two patients with cutaneous T cell lymphoma, allowing us to obtain a partial response in one case, whereas stability was observed in the second patient. These results demonstrate that As2O3 synergizes with ascorbic acid to induce Sézary cell death at clinically achievable concentrations, through a caspase-partially independent pathway, and provide a rationale for further in vivo studies addressing the therapeutic efficacy of As2O3 in cutaneous T cell lymphoma patients.

Aged↗

PubMed2003

Peripheral blood lymphocyte subset counts in patients with dermatomyositis: clinical correlations and changes following therapy.

Lymphocytopenia has been reported in patients with connective tissue diseases, including dermatomyositis (DM). However, the risk of infectious complications and the changes of lymphocytic subsets during treatment have been poorly investigated in these patients. We investigated the alterations of peripheral blood lymphocyte counts in patients with DM. A retrospective analysis was conducted in patients with an ascertained diagnosis of DM admitted from 1994 to 2000 in both departments of Dermatology of the Saint-Louis Hospital in Paris. All patients had a peripheral blood absolute lymphocyte count available before therapy. From an initial set of 63 patients, 47 were included in the study. The median absolute lymphocyte count was 888/mm(3) (range, 400-4,070). Low peripheral blood CD4+ and CD8+ T-cell and B-cell counts were consistent findings (median CD4+: 382/mm(3); CD8+: 211/mm(3); CD19+: 122/mm(3)). There was a significant increase in lymphocyte count after 1 month (p < 0.0001), 3-6 months (p = 0.001), and 6-12 months (p = 0.0005) of corticosteroid treatment. Infectious events, mainly pneumonia (PCP), occurred in 12 patients. Their initial lymphocyte count was lower than that of patients who did not develop infections (p = 0.0001). These results support the high prevalence of lymphocytopenia in patients with DM and emphasize the risk for opportunistic infections, mainly PCP, in these patients. Further studies are warranted to evaluate the risk/benefit balance of PCP prophylaxis in patients with DM and severe lymphocytopenia.

Adolescent↗

PubMed2002

B-cell lymphomas involving the skin associated with hepatitis C virus infection.

BACKGROUND: A role for hepatitis C virus (HCV) infection has been suggested in the pathogenesis of non-Hodgkin lymphomas (NHL). AIM: To evaluate the characteristics of cutaneous lymphomas occurring in HCV-infected patients, and to investigate the hypothesis of a direct infection of malignant cells. METHODS: Three patients showing a positive serology for HCV and a cutaneous lymphoma were studied. Analysis of HCV replication was performed using reverse transcriptase-polymerase chain reaction. The presence of HCV RNA was also assessed in biopsies from lesional skin and in a lymph node in one case. Genotype characterization was carried out on the basis of LiPA genotyping assays using viral genomic amplification products. RESULTS: Cutaneous NHL occurred in patients with chronic and replicative HCV infection, and exhibited a B-cell phenotype (one diffuse large B-cell lymphoma, one follicular lymphoma, and one marginal zone B-cell lymphoma). All patients were male; two of them were former intravenous drug abusers, while the HCV contamination route was unknown in the third. The search for HCV RNA in the skin and in lymph nodes yielded negative results. HCV genotypes were 1b in two cases and 3a in one case. CONCLUSION: These results show that a subset of B-cell cutaneous NHL is associated with a replicative HCV infection and suggest that HCV is not involved in lymphomagenesis through a direct infection of malignant cells. The ability of HCV to trigger a chronic B-cell lymphoproliferation could represent another mechanism, while a fortuitous association cannot be excluded.

Adult↗

PubMed2002

Paraneoplastic acral vascular syndrome: epidemiologic features, clinical manifestations, and disease sequelae.

BACKGROUND: Acral vascular syndromes associated with malignancy have rarely been reported. OBJECTIVE: Our purpose was to assess the clinical and evolving features of paraneoplastic acral vascular syndromes. PATIENTS AND METHODS: Two cases of paraneoplastic gangrene are described and analyzed together with previously reported cases identified by a MEDLINE search. RESULTS: Among the 68 patients identified, 40 had gangrene, 16 had acrocyanosis, and 12 had Raynaud's phenomenon. The male to female ratio was 0.89; median age was 59 years. Fingers were affected in 94%. Adenocarcinomas were the predominant associated malignancies (41%), and metastases were observed in 41%. The acral vascular syndromes in 48% of the patients definitively regressed after tumor treatment. Forty-four percent of the patients died within 2 years. A favorable cutaneous outcome was obtained with prostacyclin infusions in 6 patients. CONCLUSION: A neoplastic origin of acral vascular syndrome should be considered in elderly patients, especially men, in the absence of usual causative conditions.

Aged↗

PubMed2002

[Arrector pili myositis in a patient with dermatomyositis: an unusual histological feature].

Dermatomyositis is a rare inflammatory disease with a difficult diagnosis on cutaneous biopsies, since alterations are often little marked and non specific. A 52-year-old patient had severe paraneoplastic dermatomyositis. He had an erythematous rash on the trunk, and erythematous papules on the back of the hands. The first histological cutaneous lesion was a striking arrector pili myositis. Arrector pili myositis has rarely been described, and is thought to be linked with Wong type eruption occurring in dermatomyositis. Arrector pili myositis might constitute an helpful sign in the diagnosis of dermatomyositis, but its specificity and sensitivity are unknown. We compared this case to four previously reported cases. In these five cases, arrector pili myositis was not associated with a particular sub-type of dermatomyositis or with poor prognosis.

Biopsy↗