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Results for “Scleroderma, Localized”

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At least 235 records · Page 13Linked to original sources
PubMed2000

Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.

OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.

Action Potentials↗
PubMed2001

Medium-dose UVA1 phototherapy in localized scleroderma and its effect in CD34-positive dendritic cells.

BACKGROUND: UVA1 radiation seems to be effective in morphea. CD34+ dendritic cells are significantly decreased in lesional skin of morphea patients. OBJECTIVE: We evaluated the therapeutic effectiveness of medium-dose UVA1 phototherapy in localized scleroderma and its effect in the number of dermal CD34+ dendritic cells in skin biopsy specimens of these patients. METHOD: Patients were irradiated with UVA1 (30 J/cm(2)) 30 times. Dermal CD34+ dendritic cells were counted before and after therapy. RESULTS: There was clinical improvement after UVA1 irradiation. Dermal CD34+ dendritic cells significantly increased after UVA1 irradiation. CONCLUSION: Medium-dose UVA1 therapy is effective in the treatment of localized morphea. Effectiveness is associated with an increase in the number of CD34+ dendritic cells in the dermis.

Adult↗
PubMedDate not supplied

Circulatory system in children with localized scleroderma.

The circulatory system was studied in 43 children (ages 3-18 years, mean 10.6 years; 32 girls, 11 boys) suffering from localized cutaneous forms of scleroderma. The following studies were undertaken: general pediatric examination, cardiologic examination including routine electrocardiogram (ECG), 24-h Holter ECG monitoring, echocardiography plus Doppler study, and a treadmill exercise test. Three children found to have congenital heart malformations were excluded from the study. Children with localized scleroderma were often lighter and shorter than their appropriate controls. The most common ECG abnormality was incomplete right bundle branch block, but other ECG and Holter abnormalities were found as well. Abnormal echocardiographic results were obtained in 16 cases. The abnormalities concerned valvar function (in all heart valves but predominantly in the mitral valve). It was found that the children suffering from scleroderma had different indices than controls for left ventricular mass index, mitral valve function and left ventricular filling. During the exercise test (conducted according to Bruce's protocol) we found no difference between patients and controls.

Adolescent↗
PubMed1994

Clinical aspects of systemic and localized scleroderma.

Continued efforts are being made to better define the clinical course, disease subsets, and predictors of outcome in scleroderma. Data suggest that the course of the skin disease is triphasic, with the most active thickening phase in the first 12 months of disease. The presence of specific autoantibodies may predict clinical course more precisely than any clinical feature. Antipolymerase I, II, and III antibodies seem specific for scleroderma and, if present, may predict aggressive disease. Early detection of lung involvement provides an opportunity to select patients who may be responsive to drug treatment. Renal crisis in scleroderma is still important and may occur in the absence of significant signs of cutaneous fibrosis. Renin plasma levels do not appear helpful in predicting renal crisis. Significant gastrointestinal reflux disease with delayed acid clearance and esophagitis is associated with aperistalsis of the lower esophagus. Evidence for widespread structural and functional abnormalities of the microvascular circulation have been reemphasized. The psychosocial impact of scleroderma has been studied, demonstrating both the importance of depression and the need for social support. The etiology of localized scleroderma remains unknown despite efforts to link these lesions to Borrelia burgdorferi infection.

Autoantibodies↗
PubMed1997

General and local scleroderma in children and dermatomyositis and associated syndromes.

Scleroderma in children virtually always manifests in the form of localized disease, whereas adult scleroderma usually manifests as systemic disease. The recent literature on childhood scleroderma is limited almost exclusively to localized disease. The treatment of this disease remains a dilemma, especially with regard to which patients should be treated, how they should be treated, and how the treatment response should be monitored. Our current therapeutic approach is reviewed. The recent literature regarding juvenile dermatomyositis is discussed. New understanding of the pathobiology of this disease should lead to improved, less toxic treatments in the future.

Child↗
PubMed1998

[Madecassol treatment of systemic and localized scleroderma].

AIM: The trial of efficacy of 6-month therapy with madecassol (tablets, ointment, powder) of patients with systemic and focal scleroderma (SS and FS). MATERIALS AND METHODS: 54 patients (49 females and 5 males) aged 15 to 70 years with scleroderma running from 3 months to 15 years entered the study. 30 patients had typical SS, 24 patients had FS. Tablets were given to 18 patients, ointment was applied in 42 patients, powder in 3 and tablets + ointment in 9 patients. Madecassol 10 mg tablets were taken 3 times a day by patients with SS and advanced FS. The ointment was preferred in ulcers and scars on fingers and toes in SS and vascular trophic lesions in FS. In active focal scleroderma the ointment was applied to the skin lesions. The ointment was used 2 times a day (in the morning and evening) for 1-6 months. Madecassol powder was employed rarely, primarily of anal and vulval lesions. RESULTS: 6-month oral course (30 mg/day) in 12 SS patients brought about a decrease of indurative lesions, hyperpigmentation (8), vascular trophic disorders (6) and improvement of general condition (5). Subjective response was good in 10 patients and corresponded to absence of progression. In progressive disease and diffuse skin lesions the drug was ineffective. The best response was obtained in local application of madecassol ointment on digital ulcers in SS. CONCLUSION: Madecassol is effective and well tolerated and therefore recommended for oral and local use in combined treatment of SS adn FS. Indications for per os utelization are: chronic or subchronic SS with limited skin involvement, advanced and/or prone to progression FS in which combined administration of the tablets and ointment is proposed.

Adolescent↗
PubMedDate not supplied

[Localized scleroderma (morphea) and septic arthritis. Clinical manifestations of Lyme borreliosis seen in El Ferrol].

Two cases of Lyme's disease seen at El Ferrol (Spain) were described. One of them developed a recurrent knee arthritis and the other had a localized sclerodermia (morphea) syndrome. Diagnosis was established by means of clinical picture and serologic tests (enzyme-linked analysis and/or indirect immunofluorescence tests). Joint involvement has often been described in patients diagnosed of having Lyme's disease in Spain, however, the relationship between morphea and borreliosis is still a matter of controversy. We believe that patients with localized sclerodermia and high significant titers of specific antibodies against B. burgdorferi should be treated with antimicrobial agents.

Adult↗
PubMed2003

Cough sensitivity in localized scleroderma with no clinical symptoms from lower airways.

Cough sensitivity is increased in patients with atopic dermatitis, although they have no clinical symptoms from the lower airways. In the present study we examined the cough sensitivity to capsaicin in patients, who had no clinical respiratory symptoms, with sclerodermia localized to the skin. Cough sensitivity was defined as the lowest capsaicin concentration, which evokes 2 or more coughs. Twelve patients and 12 healthy matched volunteers, as a comparison group, inhaled deep breaths (2 L) of a capsaicin aerosol in doubled concentrations (from 0.02 to 200 micromol/L). Cough sensitivity, expressed as a geometric mean (95% CI) of capsaicin concentration, was 0.15 micromol/L (0.04 to 0.56) in the patients with localized sclerodermia and 4.96 micromol/L (2.50 to 9.85) in controls, which made a significant difference towards higher cough sensitivity in sclerodermia, respiratory symptom-free patients. Thus, disease processes localized outside the respiratory tract may have surreptitious pulmonary manifestation that is brought to light by the capsaicin cough test.

Adult↗
PubMed1984

Localized scleroderma (morphoea): thickness of sclerotic plaques as measured by 15 MHz pulsed ultrasound.

The thickness of morphoea plaques was measured by A-mode ultrasound and compared to regional control measurements in the same individuals. The thickness of morphoea plaques was increased by 18-310% in 17 patients with one or a few morphoea plaques (p less than 0.01), and by 13-145% in 6 patients with generalized morphoea (p less than 0.05). The increase in thickness of morphoea plaques was local confined to the plaques. Ipsilateral and contralateral control measurements were not different, and measurements in a standard region (forearm) were not different from those in a group of healthy controls matched for sex and age. Plaques of clinically 'advanced' scleroderma were more thickened (p less than 0.01) than plaques of 'slight' scleroderma. The relative increase in thickness was larger (p less than 0.01) in skin with a habitual thickness of 0.8-1.1 mm. The habitual skin thickness on the extremities (mean 1.0 mm) was less (p less than 0.01) than on the trunk (mean 1.5 mm), and, consistently, plaques with 'advanced' scleroderma were more frequent (p less than 0.05) on the extremities. Ultrasound measurement of skin thickness was accurate with SD form 0.05-0.09 mm and coefficients of variation from 3-7% in reproducibility studies of typical morphoea plaques as well as normal appearing skin.

Abdomen↗