Approximately 20% of patients with the limited form of scleroderma will develop pulmonary hypertension which is generally a late stage fatal complication. Why pulmonary hypertension occurs in this subset of patients is unknown and it has not been possible to predict which patients are at risk. Nailfold capillary dilatation, distortion and drop occurs universally in patients with scleroderma and...

BACKGROUND Previous studies have suggested an increased risk of cancer among patients with scleroderma. OBJECTIVE To study a population based cohort of patients with scleroderma in South Australia. METHODS Subjects with scleroderma were identified from the South Australian Scleroderma Registry established in 1993. All subjects on the scleroderma registry were linked to the South Australian ...

OBJECTIVES To evaluate the prevalence and clinical significance of cathepsin G antibodies in patients affected with systemic sclerosis (SSc, scleroderma). METHODS 115 patients affected by SSc, 55 (47,8%) with diffuse scleroderma (dSSc) and 60 (52,2%) with limited scleroderma (lSSc), were tested for cathepsin G antibodies by ELISA method. Moreover these sera were evaluated by indirect immunofl...

Lichen sclerosus et atrophicus and limited systemic scleroderma (acrosclerosis) are inflammatory skin diseases that ultimately evolve into two distinct modes of atrophic scar formation, but which can easily be confused clinically. They are very rarely associated. The literature has reported cases in which lichen sclerosus was associated with various forms of scleroderma, but often with localize...

Systemic sclerosis (scleroderma) is a rare fetal autoimmune disease of connective tissue leading to skin fibrosis as well as internal organ involvement (1). An annual incidence rate of 2-20 and prevalence rate of 100-300 per million population have been estimated for scleroderma with a mean survival of 12 yr from diagnosis (2-4). Moreover, there is a considerable preponderance of female gender ...

Scleroderma, or progressive systemic sclerosis (PSS), an autoimmune rheumatic condition affecting the connective tissues, has a profound impact on oral health. Common orofacial findings include xerostomia, gastroesophageal reflux disease and limited mouth opening. This review article describes scleroderma, or PSS, and its various manifestations. The features of CREST syndrome and morphea are re...

Sir, Hypertrichosis, an increase in non-androgen-modulated hair on the body, may be congenital or acquired, localized or generalized. It may be limited to cosmetic signi®cance or may represent a cutaneous sign of underlying systemic disease (1). There are a few reports of hypertrichosis on the indurated skin in melorheostotic scleroderma (2 ± 4). We describe here a 12-year-old Korean boy with l...

Scleroderma is an autoimmune disease affecting the skin and sometimes internal organs of body. There are two types scleroderma, localized scleroderma or systemic. The most common type in children it scleroderma.

OBJECTIVES There have been many studies suggesting that localized scleroderma has a strong autoimmune background, although the lesions are usually limited to the skin and subcutaneous tissue. Here we summarize previous data on the autoimmunity of localized scleroderma, mostly published in the last two decades, because there has not been a review paper summarizing autoimmunity in this disorder. ...