Dermatomyositis

Published on 05/03/2015 by admin

Filed under Dermatology

Last modified 05/03/2015

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Dermatomyositis

Dermatomyositis is an autoimmune connective tissue disease (AI-CTD) that may overlap with other AI-CTDs, in particular systemic sclerosis. It may be triggered by outside factors, most commonly malignancy (e.g. breast cancer, ovarian cancer) and occasionally drugs (e.g. ‘statins’) or rarely infectious agents (e.g. picornavirus).

Bimodal age distribution (juvenile – mean 8 years of age; adult – mean 52 years of age); female predominance.

Often clinical and laboratory evidence of proximal inflammatory myopathy (the term polymyositis is used when the disease affects muscle only; Table 34.1); patients may report difficulty combing hair or rising from a sitting position.

Pathogeneses of dermatomyositis and polymyositis are different (humoral immunity versus cell-mediated immunity, respectively).

May affect the skin only (amyopathic dermatomyositis, formerly termed dermatomyositis sine myositis; see Table 34.1); Fig. 34.1 outlines an approach to the diagnosis of this form of dermatomyositis.

Characteristic cutaneous findings include a violaceous hue of the upper eyelids with periorbital edema (Fig. 34.2) and nailfold telangiectasias (Fig. 34.3), as well as the cutaneous findings outlined in Table 34.2 and represented in Fig. 34.4A; these may precede systemic manifestations (Fig. 34.4B).

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