Dermatomyositis: A diagnostic dilemma

Silva, Diego M. Da; Patel, Basil; Werth, Victoria P.

doi:10.1016/j.jaad.2017.12.074

Cited by 30 publications

(38 citation statements)

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“…There have been several multidisciplinary attempts to come up with diagnostic and classification criteria to characterize better this skin‐predominant form of DM, especially as the risk of internal malignancy and interstitial lung disease is present among patients with purely cutaneous findings . Despite these efforts, a significant number of patients with ADM have been misclassified as having lupus erythematosus, undifferentiated connective tissue disease (UCTD) or mixed connective tissue disease (MCTD) when they present with skin findings in the background of autoimmune or rheumatological complaints . The dangers of erroneous classification include the inability to screen for comorbidities, inadequate treatment and the exclusion of patients from clinical research and experimental trials …”

Section: Bohan and Peter's Diagnostic Criteria For Dermatomyositis (Dm)mentioning

confidence: 99%

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

et al. 2019

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Background Diagnostic criteria are used to identify a patient having a 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 (IIMs). To address this, several criteria were proposed to define ADM either as a distinct disease entity or as a subset of the spectrum of IIMs. Objectives To discuss the diagnosis and classification of ADM and to assesses the available criteria in identifying cases of ADM and/or distinguishing it from dermatological mimickers such as lupus erythematosus. Methods We conducted an extensive literature search using the PubMed database from June 2016 to August 2018, using the search terms 'amyopathic dermatomyositis', 'diagnosis' and 'classification'. Results The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria, which are the only validated classification criteria for adult and juvenile IIM and their major subgroups, include three cutaneous items (G€ ottron sign, G€ ottron 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 dermatomyositis. However, about 25% of the population with ADM do not meet two out of the three skin features and are misdiagnosed or classified as having a different disease entity, most commonly lupus erythematosus. Conclusions These gaps rationalize the continuous assessment and improvement of existing criteria and/or the development of validated, separate and skin-focused criteria for DM. What's already known about this topic?• The diagnosis and classification of amyopathic dermatomyositis (ADM) have not been recognized by most existing criteria for idiopathic inflammatory myopathies (IIMs).• The latest validated European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria perform well in capturing the majority of the ADM population using three skin variables: G€ ottron sign, G€ ottron papules and heliotrope rash.• However, about 25% of patients with ADM do not meet two out of the three skin features and fail the classification.What does this study add?• The gaps in the existing classification and diagnostic criteria for IIMs rationalize the continuous assessment and improvement of existing criteria and/or the development of validated and skin-focused criteria for DM. Diagnosis and classification of amyopathic dermatomyositis, J.S.S. Concha et al. 1003Heliotrope rash, G€ ottron papules, G€ ottron sign Heliotrope rash (blue-purple discoloration on the upper eyelids with or without associated oedema) Erythematous rash on the face, neck, anterior chest (V-sign) or back and shoulders (shawl sign), knees, elbows and malleoli Mechanic's hands (rough and cracked lateral and palmar areas of the fingers) Photosensitivity and pruritus

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Section: Bohan and Peter's Diagnostic Criteria For Dermatomyositis (Dm)mentioning

confidence: 99%

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

et al. 2019

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“…In addition, there can be interdisciplinary challenges among rheumatologists, neurologists and dermatologists in classifying these patients given the nuances of skin findings in DM . It is not surprising that a retrospective study showed that 55·6% of patients had an alternate diagnosis prior to ultimately being diagnosed as having DM, with a median time delay from presentation to diagnosis of about 15 months . Altogether, 37·2% of those alternate diagnoses were either systemic lupus erythematosus or cutaneous lupus erythematosus (CLE) .…”

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confidence: 99%

“…Dermatomyositis (DM) is a heterogeneous autoimmune inflammatory disease that can be challenging to classify, particularly in patients who present predominantly with cutaneous disease. 1 Unfortunately, skin-predominant DM has historically been left out of several classification criteria schemata, [2][3][4] which has resulted in improper classification of patients and a delay in appropriate treatment and screenings. 5 In addition, there can be interdisciplinary challenges among rheumatologists, neurologists and dermatologists in classifying these patients given the nuances of skin findings in DM.…”

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confidence: 99%

“…1 Altogether, 37Á2% of those alternate diagnoses were either systemic lupus erythematosus or cutaneous lupus erythematosus (CLE). 1 These findings have major implications, as misclassifying patients can result in substandard screening for DM-associated findings, such as lung disease or cancer, 6 as well as exclusion of these patients from clinical trials or translational studies. 5 In an effort to improve existing classification criteria, a multidisciplinary collaboration, named the International Myositis Classification Criteria Project, has produced new European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for idiopathic inflammatory myopathies, including clinically amyopathic DM (CADM).…”

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Developing classification criteria for skin‐predominant dermatomyositis: the Delphi process

et al. 2019

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Summary Background The European League Against Rheumatism/American College of Rheumatology classification criteria for inflammatory myopathies are able to classify patients with skin‐predominant dermatomyositis (DM). However, approximately 25% of patients with skin‐predominant DM do not meet two of the three hallmark skin signs and fail to meet the criteria. Objectives To develop a set of skin‐focused classification criteria that will distinguish cutaneous DM from mimickers and allow a more inclusive definition of skin‐predominant disease. Methods An extensive literature review was done to generate items for the Delphi process. Items were grouped into categories of distribution, morphology, symptoms, antibodies, histology and contextual factors. Using REDCap™, participants rated these items in terms of appropriateness and distinguishing ability from mimickers. The relevance score ranged from 1 to 100, and the median score determined a rank‐ordered list. A prespecified median score cut‐off was decided by the steering committee and the participants. There was a pre‐Delphi and two rounds of actual Delphi. Results There were 50 participating dermatologists and rheumatologists from North America, South America, Europe and Asia. After a cut‐off score of 70 during the first round, 37 of the initial 54 items were retained and carried over to the next round. The cut‐off was raised to 80 during round two and a list of 25 items was generated. Conclusions This project is a key step in the development of prospectively validated classification criteria that will create a more inclusive population of patients with DM for clinical research. What's already known about this topic? Proper classification of patients with skin‐predominant dermatomyositis (DM) is indispensable in the appropriate conduct of clinical/translational research in the field. The only validated European League Against Rheumatism/American College of Rheumatology criteria for idiopathic inflammatory myopathies are able to classify skin‐predominant DM. However, a quarter of amyopathic patients still fail the criteria and does not meet the disease classification. What does this study add? A list of 25 potential criteria divided into categories of distribution, morphology, symptomatology, pathology and contextual factors has been generated after several rounds of consensus exercise among experts in the field of DM. This Delphi project is a prerequisite to the development of a validated classification criteria set for skin‐predominant DM.

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“…Recent work by Dr Victoria Werth, has shown that up to 26% of patients with ADM still fail to meet the diagnostic criteria set forth in the 2017 EULAR/ACR IIM classification . Additional work has also shown a significant percentage of patients with skin‐predominant ADM are misdiagnosed as having cutaneous lupus erythematosus . Failure to appropriately diagnose these patients as having DM is concerning for several reasons.…”

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confidence: 99%

The skin myositis Delphi group puts the details in ‘skin‐predominant’ dermatomyositis

Fernandez

2019

Br J Dermatol

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Dermatomyositis: A diagnostic dilemma

Cited by 30 publications

References 8 publications

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

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

Developing classification criteria for skin‐predominant dermatomyositis: the Delphi process

The skin myositis Delphi group puts the details in ‘skin‐predominant’ dermatomyositis

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