Objective: To evaluate the efficacy of pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy in patients with severe localized scleroderma (LS). Design: A prospective, nonran...Objective: To evaluate the efficacy of pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy in patients with severe localized scleroderma (LS). Design: A prospective, nonrandomized, open pilot study. Setting: Dermatology department at a university hospital in Bochum, Germany. Patients: Fifteen patients with histologically confirmed severe LS. Interventions: Oral methotrexate (15 mg/wk) combined with pulsed intravenous methylprednisolone (1000 mg for 3 days monthly) for at least 6 months. Main Outcome Measures: Treatment outcome was evaluated by means of a clinical score, 20-MHz ultrasonography, and histopathologic analysis. Safety assessment included the monitoring of adverse effects and clinical laboratory parameters. Results: One patient discontinued therapy. In most of the remaining 14 patients, significant elimination of all signs of active disease (inflammation) and remarkable softening of formerly affected sclerotic skin that resulted in a decrease of the mean±SD clinical score from 10.9±5.3 at the beginning to 5.5±2.5 at the end of therapy was observed (P< .001). Clinical improvement was confirmed by histologic and ultrasonographic assessments. No serious adverse effects were noted. Conclusions: These data suggest that pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy is beneficial and safe in the treatment of patients with LS. This treatment regimen should especially be considered for severe forms of LS in which conventional treatments have failed.展开更多
Background: Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly obser...Background: Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly observed, indicating the close nature of both disorders. Objective: We sought to investigate clinical, serological, and radiographic findings in PFH from 278 patients with LS and to discuss the relationship to linear scleroderma en coup de sabre (LSCS). Method: A total of 12 patients with PFH were retrospectively evaluated on the basis of clinical, serological, and radiographic findings. Results: Five patients (42% ) presented with complete PFH, and 7 patients (58% ) with partial PFH involving either cheek or forehead. Five of the patients (42% ) had a coexisting LSCS lesion, and 3 of them (25% ) had concomitant LS of the trunk. Intraoral involvement was present in 6 cases. Neurological involvement was common, in particular, epileptic seizures. Serologic investigations showed neither evidence for infection with Borrelia burgdorferi nor any other indication of underlying systemic immunological disorders. Limitations: There were a relatively small number of patients in a retrospective study. Conclusion: The reported cases suggest a close relationship between PFH and LSCS. PFH might appear as two different subtypes,one involving cutaneous structures and presenting with clinical features similar to LSCS, one being strictly restricted to subcutaneous structures, primarily affecting the cheek area. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. Magnetic resonance imaging should be included in the tools of standard diagnostic procedures in patients with PFH. The etiologic relevance of autoimmunity as well as preceding trauma should be investigated in larger collective studies.展开更多
Background. Psoralen ultraviolet A (PUVA) is the standard photo(chemo)therapeutic regimen for patients suffering from subacute prurigo (SP). Hypothesis. Regarding efficacy, bath PUVA is not superior to medium-dose ult...Background. Psoralen ultraviolet A (PUVA) is the standard photo(chemo)therapeutic regimen for patients suffering from subacute prurigo (SP). Hypothesis. Regarding efficacy, bath PUVA is not superior to medium-dose ultraviolet-A1 (MD-UVA1) and narrowband ultraviolet-B (NB-UVB), which may be considered the new photo(chemo)therapeutic options for SP. Methods. We performed a prospective randomised, controlled, three-arm photo(chemo)therapeutic study. Patients suffering from histopathologically proven SP with a clinical score (PIP score; papules, infiltration and pruritus) of at least 5 points were enrolled into the study. Treatment with bath PUVA was performed 4 times weekly and MD-UVA1 and NB-UVB 5 times weekly. Photo(chemo)therapy was administered over a 4-week period. Outcome measure was the severity of SP investigated by means of the PIP score after 4 weeks of therapy. Results. In total, 33 patients with SP were randomly allocated to photo( chemo)therapy. Bath PUVA (n = 9),MD-UVA1 (n = 11) and NB-UVB (n = 13) resulted in a significant reduction of the baseline PIP score as assessed on the basis of intention-to-treat (ITT) analysis (P = 0.003). However, ITT analysis revealed significantly higher PIP score reduction in patients who were treated with bath PUVA and MD-UVA1 compared with NBUVB (P < 0.01, 95% CI 1.1-3.63 and P < 0.05, 95% CI 0.42-2.70, respectively). Conclusions. Photo(chemo)therapy, including bath PUVA, MD-UVA1 and NB-UVB, appears to be an effective and safe treatment option for patients suffering from SP. UVA1 and particularly PUVA seem superior to NB-UVB in the management of SP.展开更多
文摘Objective: To evaluate the efficacy of pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy in patients with severe localized scleroderma (LS). Design: A prospective, nonrandomized, open pilot study. Setting: Dermatology department at a university hospital in Bochum, Germany. Patients: Fifteen patients with histologically confirmed severe LS. Interventions: Oral methotrexate (15 mg/wk) combined with pulsed intravenous methylprednisolone (1000 mg for 3 days monthly) for at least 6 months. Main Outcome Measures: Treatment outcome was evaluated by means of a clinical score, 20-MHz ultrasonography, and histopathologic analysis. Safety assessment included the monitoring of adverse effects and clinical laboratory parameters. Results: One patient discontinued therapy. In most of the remaining 14 patients, significant elimination of all signs of active disease (inflammation) and remarkable softening of formerly affected sclerotic skin that resulted in a decrease of the mean±SD clinical score from 10.9±5.3 at the beginning to 5.5±2.5 at the end of therapy was observed (P< .001). Clinical improvement was confirmed by histologic and ultrasonographic assessments. No serious adverse effects were noted. Conclusions: These data suggest that pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy is beneficial and safe in the treatment of patients with LS. This treatment regimen should especially be considered for severe forms of LS in which conventional treatments have failed.
文摘Background: Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly observed, indicating the close nature of both disorders. Objective: We sought to investigate clinical, serological, and radiographic findings in PFH from 278 patients with LS and to discuss the relationship to linear scleroderma en coup de sabre (LSCS). Method: A total of 12 patients with PFH were retrospectively evaluated on the basis of clinical, serological, and radiographic findings. Results: Five patients (42% ) presented with complete PFH, and 7 patients (58% ) with partial PFH involving either cheek or forehead. Five of the patients (42% ) had a coexisting LSCS lesion, and 3 of them (25% ) had concomitant LS of the trunk. Intraoral involvement was present in 6 cases. Neurological involvement was common, in particular, epileptic seizures. Serologic investigations showed neither evidence for infection with Borrelia burgdorferi nor any other indication of underlying systemic immunological disorders. Limitations: There were a relatively small number of patients in a retrospective study. Conclusion: The reported cases suggest a close relationship between PFH and LSCS. PFH might appear as two different subtypes,one involving cutaneous structures and presenting with clinical features similar to LSCS, one being strictly restricted to subcutaneous structures, primarily affecting the cheek area. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. Magnetic resonance imaging should be included in the tools of standard diagnostic procedures in patients with PFH. The etiologic relevance of autoimmunity as well as preceding trauma should be investigated in larger collective studies.
文摘Background. Psoralen ultraviolet A (PUVA) is the standard photo(chemo)therapeutic regimen for patients suffering from subacute prurigo (SP). Hypothesis. Regarding efficacy, bath PUVA is not superior to medium-dose ultraviolet-A1 (MD-UVA1) and narrowband ultraviolet-B (NB-UVB), which may be considered the new photo(chemo)therapeutic options for SP. Methods. We performed a prospective randomised, controlled, three-arm photo(chemo)therapeutic study. Patients suffering from histopathologically proven SP with a clinical score (PIP score; papules, infiltration and pruritus) of at least 5 points were enrolled into the study. Treatment with bath PUVA was performed 4 times weekly and MD-UVA1 and NB-UVB 5 times weekly. Photo(chemo)therapy was administered over a 4-week period. Outcome measure was the severity of SP investigated by means of the PIP score after 4 weeks of therapy. Results. In total, 33 patients with SP were randomly allocated to photo( chemo)therapy. Bath PUVA (n = 9),MD-UVA1 (n = 11) and NB-UVB (n = 13) resulted in a significant reduction of the baseline PIP score as assessed on the basis of intention-to-treat (ITT) analysis (P = 0.003). However, ITT analysis revealed significantly higher PIP score reduction in patients who were treated with bath PUVA and MD-UVA1 compared with NBUVB (P < 0.01, 95% CI 1.1-3.63 and P < 0.05, 95% CI 0.42-2.70, respectively). Conclusions. Photo(chemo)therapy, including bath PUVA, MD-UVA1 and NB-UVB, appears to be an effective and safe treatment option for patients suffering from SP. UVA1 and particularly PUVA seem superior to NB-UVB in the management of SP.
