Skin Involvement in Dermatomyositis

Beth Santmyire-Rosenberger, Elizabeth M. Dugan


Curr Opin Rheumatol. 2003;15(6) 

In This Article

Abstract and Introduction


Purpose of Review: Dermatomyositis (DM) is a rare multisystem autoimmune disorder of adults and children that primarily affects skin and skeletal muscle. Classification systems of dermatomyositis, polymyositis, and the other idiopathic inflammatory myopathies focus primarily on features of muscle involvement. However, cutaneous disease does not always parallel muscle disease in its onset, activity, or response to therapy. This review will describe the distinct cutaneous clinical and histopathologic presentation of DM and the relation between these cutaneous findings, pathogenesis of DM, and serological subsets of DM.
Recent Findings: This review discusses recent findings that have begun to elucidate the pathogenesis of DM, including polymorphism of tumor necrosis factor-α 308A allele and maternal fetal microchimerism. The recent description of other systemic diseases and drugs causing DM-like eruptions and the recognition that DM can resemble other common dermatoses highlights the need for a cutaneous biopsy to diagnose and distinguish the cutaneous features of DM. Once diagnosed, a number of noninvasive imaging modalities and new cutaneous assessment instruments can be used to follow and evaluate patients with DM.
Summary: Recognition of cutaneous and histopathologic findings in DM is essential for prompt and accurate diagnosis and treatment of DM.


Dermatomyositis (DM) is a rare multisystem autoimmune disorder that primarily affects skin and skeletal muscle. It affects children and adults of both genders and of all ethnic groups, with a predilection for females. This review will focus on cutaneous involvement in DM. A clear recognition of the cutaneous findings is essential for prompt and accurate diagnosis of DM, especially in cases in which cutaneous manifestations are the initial or only findings. Dermatologists play a primary role in the diagnosis and care of DM. In one recent retrospective study of juvenile DM, 25% of patients complained only of a rash on initial presentation, and 38% of patients presented to a dermatologist first, regardless of other symptoms.[1] Cutaneous manifestations of DM may precede the onset of myositis by at least several months and in some reports for up to 2 years or more.[2] In addition, skin involvement can be the most active or severe component of DM, failing to respond to therapeutic interventions that are adequate for myositis and other systemic involvement. Therefore, we believe that it is critical to recognize and evaluate cutaneous manifestations of DM independent of skeletal muscle disease activity.


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