BACKGROUND Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. METHODS We ...

Increasing interest in the vascular features of scleroderma has led to the hypothesis that the blood vessel is the major target tissue and that the endothelial cell is the principal cell target. Useful observations stemming from the vascular hypothesis include the use of microvascular abnormalities in the early detection of the patient destined to develop classical scleroderma, the discovery of...

BACKGROUND Scleroderma is a chronic connective tissue disease of unknown etiology. Vitamin D and parathyroid hormone (PTH) that play particular functions in calcium and phosphate homeostasis may be involved in the etiology of this disorder. Klotho, the co-receptor of the fibroblast growth factor 23 (FGF-23), can interfere with calcium and phosphate metabolism. The purpose of this study was to e...

Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenido...

BACKGROUND β-thymosins play roles in cytoskeleton rearrangement, angiogenesis, fibrosis and reparative process, thus suggesting a possible involvement in the pathogenesis of systemic sclerosis. The aim of the study was to investigate the presence of thymosins β4, β4 sulfoxide, and β10 in bronchoalveolar lavage fluid of scleroderma patients with interstitial lung disease and the relation of thes...

INTRODUCTION Scleroderma is a progressive fibrotic disorder of connective tissue which can present multiple anesthetic challenges to anesthetists, especially airway management. Awake intubation with fiberoptic bronchoscope is widely accepted and implemented for progressive systematic scleroderma patients. With the development and improvement of intubation devices these years, there is no report...

Objective. We report a rare case of keloidal scleroderma and provide an analysis of similar cases. Results. A 41 year-old woman presented with dark brown, indurated, exophytic nodules over the chest along with smaller hyperpigmented plaques scattered over the abdomen, with concomitant sclerodactyly.The clinical, laboratory, and pathological findings were consistent with a diagnosis of keloidal ...

OBJECTIVES To describe the frequency, extent, and nature of microvascular lesions in patients with scleroderma by means of capillaroscopy and capillaroscopically guided nail fold biopsy, and to determine the diagnostic value of the two methods and the pathophysiological significance of the lesions described. METHODS A cohort study was made of 24 consecutive patients with scleroderma and 10 he...

BACKGROUND Scleroderma renal crisis is an important complication of scleroderma (systemic sclerosis) that is associated with significant morbidity and mortality. On the other hand, hyponatremia has never been reported in patients with scleroderma renal crisis. CASE PRESENTATION A 66-year-old man with scleroderma was admitted to our hospital for an evaluation of renal dysfunction and extreme h...