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Diagnostic Criteria

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Dermatomyositis

The purpose of classification criteria for idiopathic inflammatory myopathies is to define homogenous groups of patients [1]. These criteria can be used in studies that aim to increase the knowledge about disease mechanisms and to improve the treatment in disorders in which etiology is unknown. Great progress has been made during the past 30 years in characterizing the clinical features of IIMs. In 1975 Bohan and Peter, focusing upon non-neurogenic muscle involvement, suggested five diagnostic criteria for the diagnosis of PM/DM, which today are an accepted standard [2, 3] (Table 4.1). The presence of distinguishing skin features, including heliotrope rash, Gottron's papules, and characteristically distributed macular erythemas, is the fifth criterion. Although these are the most typical cutaneous manifestations, skin findings of DM can be heterogeneous, and these hallmark cutaneous lesions may not be evident at the time of presentation [4].

The presence of three or four criteria for myositis plus the cutaneous rash in a patient defines a definitive diagnosis for DM, and the availability of four criteria without the skin rash provides the same for PM. Bohan and Peter outlined a systemic way to describe the probability of having the diseases as definite, probable, and possible. The patients who fulfill four criteria have definite PM/DM, those who cover three criteria have probable PM/ DM, and patients with two criteria — possible PM/DM. Cutaneous eruption must be one of the stated number of criteria in patients with definite, probable, or possible DM.

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(2009). Diagnostic Criteria. In: Dermatomyositis. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-79313-7_4

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