A 52-year-old woman presented with a large partially yellow and erythematous tumor on her right temple. She reported that it had grown over the last 4 years. Regional lymph nodes were impalpable. A punch biopsy showed eosinophilic material in the dermis and subcutis. Immunohistochemistry showed positive staining for kappa and lambda light chains. Electron microscopy showed the typical amyloid f...

systemic sclerosis is a generalized disorder of connective tissue, in which the pattern of disease extent, progression and outcome is heterogenous. to determine clinical features, disease extent and progression, we studied our patients in two phases of disease early (the first 3 years) and late phases (after 6 years of disease). 19 patients had diffuse cutaneous and 34 patients had limited cuta...

BACKGROUND For the serological diagnosis of systemic autoimmune rheumatic diseases, a two-tier approach starting with sensitive antinuclear antibody (ANA) detection by indirect immunofluorescence (IIF) on HEp-2 cells followed by characterization of positive findings with different immunoassays is recommended. To overcome drawbacks of this approach, we developed a novel technique allowing the co...

Nephrogenic systemic fibrosis (NSF) is a severe iatrogenic disease that affect patients with impaired renal function exposed to gadolinium-based contrast agents. Clinically, symptoms develop within days or weeks after the exposure and mimic a scleromyxedema. The causal relationship between use of gadolinium-based contrast agents and NSF led to develop clinical guidelines aiming to limit the use...

Scleredema adultorum or Buschke's scleredema is a rare disorder that belongs to the group of mucinoses. Diffuse, sudden swelling, hardening and induration of the skin can occur in children and younger women as well as in older men. The dermis is thickened because of the increased collagen glycosylation, like that in diabetic stiff skin syndrome. The face is most often involved. In older persons...

INTRODUCTION Systemic sclerosis is a systemic connective tissue disease with variable cutaneous presentations. Although pigmentary disturbances have been described in systemic sclerosis, a reticulate hyperpigmentation has only been reported in one case of systemic sclerosis to date. CASE PRESENTATION We describe a previously healthy 51-year-old Thai woman who presented with a reticulate hyper...

OBJECTIVE To determine serum concentrations of soluble P-selectin glycoprotein ligand-1 (sPSGL-1) and its clinical associations in patients with systemic sclerosis. METHODS Serum sPSGL-1 concentrations from 65 patients with systemic sclerosis were examined by enzyme linked immunosorbent assay. In a retrospective longitudinal study, 177 sera from 35 patients with systemic sclerosis were analys...

PURPOSE Systemic sclerosis is a life-threatening autoimmune disease characterized by vasculopathy, which results in myocardial involvement in an extremely high percentage of patients. Nevertheless, there have been no large-scale epidemiological studies about the risk of acute myocardial infarction in patients with systemic sclerosis. The aims of this study were to evaluate the hazard ratio (HR)...

Introduction Although it is a rare disorder, scleromyxedema (lichen myxedematosus) is one of the most common forms of primary cutaneous mucinosis. This condition is a generalized form of dermal mucin deposition characterized by waxy papules that progresses to indurated and thickened skin and is often associated with monoclonal gammopathy, particularly of the immunoglobulin G type. Lichen myxede...