• Jeffrey P. Callen


Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies (IIM) (Callen 2000; Plotz et al. 1995; Targoff 1991). In 1975, Bohan and Peter published a classical article that first suggested a set of criteria to aid in the diagnosis and classification of DM and polymyositis (PM). Four of the five criteria related to the muscle disease: 1) progressive proximal symmetrical weakness 2) elevated muscles enzymes, 3) an abnormal electromyogram and 4) an abnormal muscle biopsy, while the fifth was the presence of compatible cutaneous disease. It was felt that DM differed from PM only by the presence of cutaneous disease. Recent studies of the pathogenesis of the myopathy have been controversial, some suggesting that the myopathies in DM and PM are pathogenetically different with DM being due to a vascular inflammation (Kuru et al. 2000) while other studies of cytokines suggest that the processes are similar (Wanchu et al. 1999; Shimizu et al. 2000; Sugiura et al. 2000; Nyberg et al. 2000). There has been a renewed interest in the pathogenetic mechanisms involved in the myopathy with recent studies revealing abnormal levels of nitric oxide, elevation of circulating tumor necrosis factor receptors, elevated soluble CD40 expression, and increased expression of interleukin 1-alpha within the muscle. The pathogenesis of the cutaneous disease is poorly understood.


Muscle Disease Inclusion Body Myositis Cutaneous Manifestation Idiopathic Inflammatory Myopathy Cutaneous Lupus Erythematosus 
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© Springer-Verlag Wien 2001

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  • Jeffrey P. Callen

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