Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by the presence of skin lesions and inflammation of skeletal muscles; however, this feature may be absent in amyopathic DM. DM is a rare disease, occurring at any age, and has two peaks of incidence: one in childhood between 5 and 15 years of age and one in adulthood between 40 and 60 years, with a female preponderance. DM has been associated with malignancy; therefore, every newly diagnosed patient should undergo screening investigations, but evidence-based guidelines on their extension are lacking. The etiopathogenesis is still unclear, but a range of factors such as genetic predisposition, environment triggers, and immune- and non-immune-mediated mechanisms play a role in the development of the disorder. Some dermatological findings and new discoveries on immunological mechanisms in DM, including the association of certain autoantibodies with cancer, can be helpful in clinical practice. Therapy should consider both skin and muscle or internal organ involvement. Photoprotection, including the use of antimalarial drugs, plays an important role in the therapeutic approach of skin involvement. Treatment of muscle and internal organ involvement is based on systemic corticosteroids, alone or in association with disease-modifying antirheumatic drugs. Intravenous immunoglobulins have been demonstrated to be effective as second-line therapy. Rituximab is an emerging treatment for difficult-to-treat DM, both in adults and children. In this review article, we discuss DM subforms, focusing on their dermatological manifestations that can be classified as pathognomonic, characteristic, compatible, less common, rare, recently-described, and non-specific skin lesions.

Cutaneous Manifestations of Dermatomyositis: a Comprehensive Review.

The differential expression of VEGF, VEGFR-2, and GLUT-1 proteins in disease subtypes of systemic sclerosis

Objectives. To investigate a large cohort of children with juvenile dermatomyositis (JDM), and those with JDM–scleroderma (JDM–SSc) overlap, using detailed serological analysis, HLA class II genotyping and clinical characterization. Methods. Children (114) with JDM were recruited, and clinical data collected, through the JDM National Registry and Repository (UK and Ireland). Sera were assayed for ANA using standard immunofluorescence techniques and specific antibodies characterized using ELISA, immunodiffusion and radioimmunoprecipitation. Patients and controls (n ¼ 537) were genotyped at the HLA-DRB1 and DQB1 loci, and then the DQA1 locus data was derived. Results. Over 70% of the patients were ANA-positive. Clear differences in serological and genetic data were demonstrated between JDM and JDM–SSc overlap groups. Strong associations were seen for HLA-DRB1 � 03 (all cases vs controls, Pcorr ¼ 0.02; JDM–SSc vs controls, Pcorr ¼ 0.001) and HLA-DQA1 � 05 (all cases vs controls, Pcorr ¼ 0.01; JDM–SSc vs controls, Pcorr ¼ 0.005). The frequency of the HLA-DRB1 � 03-DQA1 � 05-DQB1 � 02 haplotype was significantly increased in the JDM–SSc (P ¼ 0.003) and anti-PM-Scl antibody (P ¼ 0.002) positive groups. All anti-U1-RNP antibody-positive patients had at least one copy of HLA-DRB1 � 04-DQA1 � 03-DQB1 � 03 haplotype. Associations were observed between serology and specific clinical features. Conclusions. We present clinical data, HLA genotyping and serological profiling on a large cohort of JDM patients and a carefully characterized subset of patients with JDM–SSc overlap. The results confirm known HLA associations and extend the knowledge by stratification of data in serological and clinical subgroups. In the future, a combination of serological and genetic typing may allow for better prediction of clinical course and disease subtype in JDM.

http://myonet.eu/wp-content/uploads/2013/05/17.-Wedderburn-et-al.-2007.pdf

HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis–scleroderma overlap

The clinical cardiac manifestations most frequently reported in idiopathic inflammatory myopathies, myositis, are congestive heart failure, conduction abnormalities, that may lead to complete heart block and coronary artery disease. Although clinically overt cardiac involvement is rarely reported in myositis patients, subclinical manifestations are frequently observed and are predominated by conduction abnormalities and arrhythmias detected by ECG. Furthermore, cardiovascular manifestations constitute a major cause of death in myositis, thus cardiac involvement maybe overlooked in these patients. Also children with juvenile dermatomyositis may develop cardiac involvement although the frequency seems to be low. The underlying pathophysiologic mechanisms that may cause cardiac manifestations could involve myocarditis and coronary artery disease as well as involvement of the small vessels of the myocardium. In patients with mixed connective tissue disease (MCTD) clinically significant cardiac involvement is ...

Cardiac involvement in autoimmune myositis and mixed connective tissue disease.

Inflammatory myopathies in children.

Juvenile dermatomyositis is a chronic inflammatory illness of unknown etiology that affects primarily muscle and skin. It has an incidence of 2-3 per 1,000,000, per year. The disease can affect other organ systems, including the gastrointestinal tract, lungs, and heart. In addition, calcinosis is seen in one-third of patients. The mainstay of therapy is corticosteroids; some children require additional immunosuppressive agents because of corticosteroid resistance or intolerance. Functional outcomes have become good with modern treatments, but the disease remains chronic in a large number of children and sequelae are often seen.

Clinical outcomes in juvenile dermatomyositis.

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Clinical outcomes in juvenile dermatomyositis.