Cytotoxic T Lymphocytes
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Cancer and Dermatomyositis George I. Varghese1, Joshua Weaver2 and Mathew C. Varghese1 1 Weill Cornell Medical College, Department of Dermatology, New York, NY, USA 2 Department of Neurology, Weill Cornell Medical College, New York, NY, USA
polymyositis (PM) and inclusion body myositis (IBM), DM is a distinct disorder with a unique pathophysiology. This entry will review the clinical signs and symptoms, workup, treatment, and prognosis of DM and its subtypes. The immunopathology and pathophysiology of DM is emphasized within the entry, as there has been a rapidly growing body of literature in these areas.
Synonyms Epidemiology Amyopathic dermatomyositis; Idiopathic inflammatory myopathy; Juvenile Dermatomyositis; Polymyositis
Definition Dermatomyositis – Inflammation of the skin and underlying muscle tissue, typically occurring as an autoimmune condition or associated with internal cancer.
Introduction Dermatomyositis (DM) is an acquired skeletal muscle disease with characteristic skin and extramuscular manifestations and is generally classified as an idiopathic inflammatory myopathy. Though DM has a wide range of phenotypic presentations and clinical overlap with other idiopathic inflammatory myopathies, including
Dermatomyositis can affect a wide age group with two peaks of onset, including 5–10 years of age and 30–50 years of age. The incidence is roughly 1 per 100,000 annually (Dalakas and Hohlfeld 2003), and it is about twice as common in women than men. There is no racial predilection.
Clinical Signs/Symptoms Given the considerable clinical overlap between the idiopathic inflammatory myopathies, neuromuscular disorders, and many connective tissue disorders, definitive diagnosis has long been a challenging issue. In 1975, Bohan and Peter created a set of diagnostic criteria for polymyositis and dermatomyositis that included clinical symptoms and signs, muscle pathology, serum biochemical markers, and electromyographic data. With the advent of more advanced
I.R. Mackay, N.R. Rose (eds.), Encyclopedia of Medical Immunology – Autoimmune Diseases, DOI 10.1007/978-0-387-84828-0, # Springer Science+Business Media New York 2014
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Cancer and Dermatomyositis
Cancer and Dermatomyositis, Fig. 1 A violaceous hue with periorbital edema is consistent with heliotrope sign of dermatomyositis (Source: Dr. George I. Varghese)
immunological research, these criteria have since been modified (Callen 2000; Dalakas and Hohlfeld 2003). Clinically, the most salient feature that distinguishes DM from other inflammatory myopathies is the skin involvement. It is often the first manifestation and, in a subset of people with DM, can be the sole manifestation as well (Callen 2000). The heliotrope rash, a violaceous macular erythema symmetrically involving the periorbital skin, with or without underlying edema, is pathognomonic for DM (Fig. 1). It may be subtle, only involving the eyelid margin. Gottron’s papules, also specific for DM, are raised violaceous papules and plaques with a scaly eruption over the bony prominences
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