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...

Scleroderma (skleros; hard, and derma; skin), is currently known as systemic sclerosis due to its progressive nature and widespread tissue involvement. It is a rare connective tissue disorder with a wide range of oral manifestations. Thickening of the skin is the hallmark of the disease. The patient education for self-care and multidisciplinary approach would be needed to manage the condition. ...

The scleroderma group of diseases is characterized by the presence of hard skin. The pathogenesis of scleroderma include excessive production of collagen and extracellular matrix by fibroblasts, not only in the skin but also in vital organs and around blood vessels with endothelial cell injury. Systemic Sclerosis (SSc) usually constitutes less than 1% of most pediatric rheumatology clinic popul...

INTRODUCTION Platelet aggregation may contribute to the pathogenesis of systemic sclerosis: following activation, platelets release significant amounts of serotonin - which promotes vasoconstriction and fibrosis, and further enhances aggregation. The C+1354T polymorphism in the exonic region of the serotonin 2A receptor gene determining the His452Tyr substitution was associated with blunted int...

Secondary mucin deposition in the skin is a common feature of lupus erythematosus and dermatomyositis. In scleroderma, it occurs uncommonly or in small amount. We describe a 7-year-old boy with progressive, linear, bound-down plaques involving the thighs, lower abdomen and back with no systemic involvement. Histopathology showed features of scleroderma with abundant mucin deposition in the reti...

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...

A case of scleroderma in a woman with carcinoid syndrome is described and the similarities between our case and those in the literature are reviewed. The carcinoid tumours were all of midgut origin and liver metastases were present. All subsequently developed fibrotic heart disease and none had clinical features or autoantibodies suggestive of systemic sclerosis. The association between carcino...

In this report, we describe the use of extracorporeal photochemotherapy in n the treatment of two patients with rapidly advancing progressive systemic sclerosis. Both patients experienced improvement in the cutaneous as well as the systemic manifestations of their disease while undergoing therapy. The potential therapeutic mechanisms are discussed.

A 36-year-old female with diffuse cutaneous systemic sclerosis (dcSSc) developed macrohematuria due to vesical telangiectasias that was responsive to diathermocoagulation of the vasal lesions. This is the first report of a patient with dcSSc and vesical telangiectasias leading to severe macrohematuria that was successfully treated with diathermocoagulation.