Biomedical subjects
Publications and source records attributed to Florence Cordoliani.
Calcifications in idiopathic calcinosis cutis are most commonly localized to one area. We herein report a 66-year-old woman with idiopathic calcinosis cutis who unusually exhibited widespread calcific deposits. In this report, we will also briefly discuss the pathogenesis, differential diagnosis, and current treatment of this disease.
BACKGROUND: Superficial venous thrombophlebitis (SVT), often perceived as benign, can coexist with hypercoagulable states. Predisposing risk factors for SVT are similar to those observed for deep venous thrombosis. Association of eosinophilia with SVT is a rare situation that can reveal neoplasia, malignant blood disorders, or vasculitis, but it has never been described in hypereosinophilic syndrome (HES). We herein describe the clinical and biological features, outcome, and response to therapy of 3 patients with SVT associated with eosinophilia that revealed HES. OBSERVATIONS: Superficial venous thrombophlebitis was the initial manifestation of HES in all 3 patients. The mean eosinophil count at diagnosis was 2.4 x 10(3)/muL. All patients received corticosteroids and anticoagulants as the initial treatment, with marked improvement of SVT and return of the eosinophil count to reference limits. All patients experienced relapse and remained dependent on corticosteroid therapy. Two patients received interferon alfa with dramatic regression of SVT, allowing a decrease in the dose of corticosteroids. CONCLUSIONS: We report, to our knowledge, the first 3 cases of SVT related to HES. Superficial venous thrombophlebitis was difficult to treat, with dependence on corticosteroid therapy and partial efficacy of anticoagulant and antiplatelet therapy. Interferon alfa was effective in preventing relapse of SVT related to HES. Mechanisms implied in this thrombogenesis are multiple and remain speculative.
BACKGROUND: Cutaneous calcification, or calcinosis cutis (CC), is found in approximately 1% of patients with end-stage renal disease (ESRD) undergoing chronic dialysis. While the pathogenesis is not well understood, it may be similar to those for medial and intimal vascular calcifications, which are actively regulated processes. OBSERVATION: In a retrospective study of 9 patients, the role of an active calcification process leading to CC was assessed by the immunohistochemical detection of osteopontin, which is a regulator of osseous and extra-osseous calcification processes. Calcinosis cutis was associated with female sex, vascular comorbidity, inconstant secondary hyperparathyroidism, and elevated levels of plasma calcium-phosphorus product. Six patients had a favorable outcome after the lowering of plasma calcium levels during dialysis or after parathyroidectomy. CONCLUSIONS: Calcinosis of the vascular media of subcutaneous vessels was the most common histologic feature and was always associated with osteopontin staining, suggesting that CC is a regulated process. Moreover, to our knowledge, extravascular staining of osteopontin in sweat glands, nerves, and macrophages was demonstrated for the first time in this study.
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.
BACKGROUND: Although systemic corticosteroids are effective against cutaneous sarcoidosis, alternative therapies are needed. OBJECTIVE: We sought to assess the efficacy and tolerance of thalidomide for cutaneous sarcoidosis. METHODS: We performed a retrospective evaluation of thalidomide (100-200 mg/d) in 12 consecutive patients with cutaneous sarcoidosis treated in a university hospital between 2000 and 2002. RESULTS: Cutaneous lesions regressed within 1 to 5 months, with an average time of 2 to 3 months for 10 patients. In all, 4 patients achieved complete responses, 6 had partial responses, and 2 had no regression. Nasopharyngeal, pulmonary neurologic, and hepatic symptoms were also attenuated. Thalidomide was well tolerated. The main adverse effect was deep vein thrombosis in 1 patient. CONCLUSION: Thalidomide efficacy and tolerance in patients with cutaneous sarcoidosis merits further evaluation in a controlled trial.