BACKGROUND Dermatomyositis is an idiopathic inflammatory myopathy that mainly affects the skin and skeletal muscle. An estimated 15% to 25% of patients have underlying tumors and some forms are exclusively cutaneous. The factors that predict disease course and prognosis in these patients have not been clearly identified. Here we report our experience through the description and analysis of a se...

Cutaneous manifestations of dermatomyositis are generally grouped as pathognomonic, characteristic, compatible, less common and rare (Table 4). The primary lesion appears as a violaceous, macular erythema with a symmetric distribution. This may progress and become poikilodermatous (atrophic with telangiectasia and pigmentary changes) and indurated (as a result of mucin deposition). Pathognomoni...

Purpose Recent studies have implicated low vitamin D levels with greater disease activity in pediatric systemic lupus erythematosus (SLE) and we have reported an association between low vitamin D levels and greater disease activity in juvenile dermatomyositis. The objective of this study was to explore whether standardized vitamin D supplementation of low levels of 25-hydroxyvitamin D [25(OH)D]...

Purpose Juvenile dermatomyositis (JDM) is a rare disease which has been difficult to evaluate objectively due to the low incidence of disease. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) initiated a multicenter observational registry to create a clinical database for the major rheumatic diseases of childhood, including JDM. Initial data from the JDM cohort (prevalent and ...

Introduction Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by proximal muscle weakness and cutaneous manifestations, typically heliotrope rash and Gottron’s papules. Previous studies have identified an increase in circulating B cells in JDM patients, but their provenance and functional characteristics have not been examined. In this study we investigated whether a recent...

Case 1 17-year-old boy referred to the Autoimmune Diseases Centre with symmetrical proximal muscle weakness, weight loss, dysphagia, periungual lesions and erythema of hands and eyelids with a year of evolution. Laboratory tests showed elevated lactate dehydrogenase, creatine kinase, transaminases and aldolase. Immunological/serological tests were negative. Capillaroscopy was compatible with Ju...

Introduction Juvenile dermatomyositis patients may experience persistent weakness, loss of bone mass, and skeletal muscle atrophy as a long-term result of drug treatment and/or disease itself. In this regard, efforts to develop new therapeutic strategies able to attenuate these adverse outcomes have been considered of clinical relevance. It has been suggested that creatine supplementation could...

Background Juvenile dermatomyositis (JDM) is thought to involve an autoimmune myositis, yet the elements of the immune response which damage muscle tissue in JDM remain unclear. Muscle tissue from early JDM shows infiltration by predominantly macrophage/myeloid cells. Traditional histopathology would suggest that these cells have a scavenger or 'repair' function; our data suggest otherwise. We ...

Introduction Biologic therapy is increasingly prescribed in rheumatologic disorders. Juvenile dermatomyositis (JDM), the most common inflammatory myopathy in children, can be challenging to manage in a subset of patients. There are multiple reports of cytokine involvement in JDM. There is a paucity of information regarding the use of biologics for JDM among pediatric rheumatology practitioners,...