Biomedical subjects
Manuelle Viguier
Publications and source records attributed to Manuelle Viguier.
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.
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.
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.
[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.