Primary localized cutaneous amyloidosis (PLCA) is a relatively rare condition characterized by amyloid de-position exclusively in the dermis without involving the internal organs. Clinically, papular, macular and tumefac-tive forms are presented. Although PLCA may sometimes overlap with collagen vascular diseases, association with scleroderma is rare. We report here two cases of PLCA developing...

Cell-mediated immunity to skin extracts was studied by the macrophage migration inhibition test, lymphocyte transformation, and direct cytotoxicity to skin fibroblasts, in normal individuals and patients with progressive systemic sclerosis. The latter included 18 individuals with diffuse scleroderma and 12 with the CREST syndrome, a variant form of systemic sclerosis in which there is more limi...

Introduction: Pulmonary arterial hypertension (PAH) is a late progressive sclerodermarelated complication, which can lead to right heart failure and cor pulmonale. Given that cardiac catheterization is a diagnostic method of choice for PAH, and considering the high risks of this method, the purpose of this study was to evaluate the relationship between serum Pro‐Brain natriuretic peptide (Pro‐B...

Hospital Physician January 2008 33 S ystemic sclerosis, or scleroderma, is a rare connective tissue disease of unknown etiology. The word scleroderma, derived from the Greek words scleros for hard and derma for skin, refers to the hallmark finding of chronic hardening and thickening of the skin that occurs in this disease. The clinical manifestations of systemic sclerosis range from localized s...

Therapy of localized scleroderma is unsatisfactory, with numerous treatments being used that have only limited success or considerable side-effects. The aim of this trial was to determine whether topical photodynamic therapy would be effective in patients with localized scleroderma. Five patients with progressive disease, in whom conventional therapies had failed, were treated by application of...

Fibrosis, characterized by excessive extracellular matrix accumulation, is a common feature of many connective tissue diseases, notably scleroderma (systemic sclerosis). Experimental studies suggest that a complex network of intercellular interactions involving endothelial cells, epithelial cells, fibroblasts and immune cells, using an array of molecular mediators, drives the pathogenic events ...

Scleroderma en coup de sabre typically presents with a cutaneous induration, but involvement of the underlying bone, eye, and brain is common. We report on 4 pediatric cases with atypical initial clinical presentations. All cases were seen at the University of California San Francisco. Patients 1 and 2 presented to the Pediatric Rheumatology Clinic with uveitis and orbital pseudotumor in Decemb...

Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been repo...

Several abnormalities of cytokines have been shown to occur in systemic scleroderma; however their correlation with clinical parameters is controversial. Since serum concentrations of cytokine receptors have been shown to correlate with inflammatory processes, including systemic sclerosis, the aim of our study was to compare serum concentrations of TNF alpha receptor type 1 with the concentrati...

primary hepatic lymphoma (phl) is rare and possibly associated with viral hepatitis and autoimmune diseases. scleroderma could exceptionally be complicated by lymphoma. we describe phl occurring in a 52-year-old female suffering scleroderma for eight years, with no history of cytotoxic or high-dose glucocorticoid therapy. ct scan, performed to work-up abdominal discomfort, constipation, and ele...