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Guidelines on dermatomyositis – excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology

The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at www.awmf.org). The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30 % of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup. Although the cutaneous manifestations in DM are variable, taken together, they represent a characteristic and crucial diagnostic criterion for DM. Approximately 5–20 % of individuals exhibit typical skin lesions without any clinically manifest muscle involvement (amyopathic DM). About 30 % of adult DM cases are associated with a malignancy. This fact, however, should not delay the treatment of severe myositis.