Received: Jan 09, 2013 Accepted: Jan 16, 2013 Ann Paediatr Rheum 2013; 2:43-49 DOI: 10.5455/apr.011620131348 Introduction Juvenile localized scleroderma ( JLS) is a childhood condition characterized by thickening of the skin. A variety of clinical subtypes have been described, differentiated by the size, shape, and location of skin involvement as well as the presence or absence of extra-cutaneo...

The recent shift to the use of stem cells mobilized by granulocyte colony-stimulating factor (G-CSF) for hematopoietic transplantation has increased chronic graftversus-host disease (GVHD), although the mechanisms of this are unclear. We have found that G-CSF invokes potent type 17 rather than type 1 or type 2 differentiation. The amplification of interleukin-17 (IL-17) production by G-CSF occu...

BACKGROUND Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear syndrome. In a number of patients, neurological affection is the medium of the disease. T...

Scleroderma is an autoimmune disease of the connective tissue characterized by fibrosis and thickening of various tissues. It can be limited to the skin or affect multiple organs, and its course ranges from slowly to rapidly progressive. Penicillamine, glucocorticoids, and other drugs are used to treat scleroderma, but none of these treatments has a high degree of efficacy. This article reviews...

Primary cardiac involvement in systemic sclerosis is common, often subclinical, and is associated with significant mortality. We report the case of a patient who developed autoimmune myocarditis at an early stage of systemic sclerosis, who completely recovered from cardiac dysfunction under optimal medical therapy for heart failure and immunosuppression. This challenging case aims at increasing...

Porphyria cutanea tarda and erythropoietic porphyria are disorders of heme synthesis that originate in the liver and bone marrow, respectively. Each is characterized by increased accumulation of uroporphyrin, I, by cutaneous photosensitivity, and in some patients by indurated plaques and scarring that resemble scleroderma. These scleroderma-like lesions occur in light-exposed and light-protecte...

A 62-year-old woman with long-standing limited scleroderma presented to the outpatient rheumatology clinic because of pain and discomfort of the nose. On physical examination a firm subcutaneous nodule was observed over the ridge of the nose (A). No other such nodule-like lesions were observed. The nature of this nodule was disclosed by plain radiographs, which clearly demonstrated a subcutaneo...

OBJECTIVE To determine the diagnostic distribution in a consecutive anti-SSA and/or anti-SSB positive population. METHODS A total of 15 937 serum samples from 10 550 consecutive patients were analysed for antinuclear antibodies (ANAs) on HEp-2 cells. Serum samples positive for ANAs were analysed by immunodiffusion and line immunoassay with recombinant SSA-Ro52, natural SSA-Ro60, and recombina...

Transforming growth factor-ß (TGF-ß) signaling is implicated in the pathogenesis of fibrosis in scleroderma or systemic sclerosis (SSc), but the precise mechanisms are poorly understood. The immediate-early gene Egr-1 is an inducible transcription factor with key roles in mediating fibrotic TGF-ß responses. To elucidate Egr-1 function in SSc-associated fibrosis, we examined change in gene expre...

BACKGROUND Cutaneous sclerosis, a process that results in hardening of the skin, is the hallmark of scleroderma and sclerodermoid disorders. Cutaneous sclerosis is usually classified as secondary or primary, depending on the presence or absence of underlying diseases. Primary cutaneous sclerosis is a feature of idiopathic inflammatory processes that are often associated with autoimmune disorder...