The diagnosis and classification of amyopathic dermatomyositis: a historical review and assessment of existing criteria

Abstract

Diagnostic criteria are used to identify a patient having the disease in a clinical setting, whereas classification criteria create a well‐defined population for research purposes. The diagnosis and classification of amyopathic dermatomyositis (ADM) have not been recognized by most existing criteria for idiopathic inflammatory myopathies (IIM). To address this, several criteria were proposed to define ADM either as a distinct disease entity or as a subset of the spectrum of IIM. The EULAR/ACR criteria which are the only validated classification criteria for adult and juvenile IIM and their major subgroups include three cutaneous items (Göttron sign, Göttron papules, heliotrope rash) to be able to classify ADM. This international and multispecialty effort is a huge step forward in the classification of skin‐predominant disease in DM. However, about 25% of the ADM population do not meet two out of the three skin features and are misdiagnosed or classified as a different disease entity, more commonly as lupus erythematosus. These gaps rationalize the continuous assessment and improvement of existing criteria and/or the development of validated, separate, and skin‐focused criteria for DM.

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