Amyopathic Dermatomyositis

Caproni, Marzia; Cardinali, Carla; Parodi, Aurora; Giomi, Barbara; Papini, Manuela; Vaccaro, Mario; Marzano, Angelo Valerio; Simone, Clara De; Fazio, M; Rebora, Alfredo; Fabbri, Paolo

doi:10.1001/archderm.138.1.23

Cited by 56 publications

(6 citation statements)

References 23 publications

(35 reference statements)

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“…An example are DM patients with characteristic muscle complaints and the hallmark skin lesions. However, DM patients may also present with muscle weakness only (DM sine dermatitis) or solely exhibit cutaneous manifestations (amyopathic DM) ( 38 ). In IIM patients, diagnosis most often requires combining data of a patient’s clinical signs with serological, pathological and genetic analyses.…”

Section: Discussionmentioning

confidence: 99%

“…An example are DM patients with characteristic muscle complaints and the hallmark skin lesions. However, DM patients may also present with muscle weakness only (DM sine dermatitis) or solely exhibit cutaneous manifestations (amyopathic DM) (38) signs with serological, pathological and genetic analyses. As much as a skilled neuropathologist is essential for the diagnosis of IIM, such an expert may be a rare find.…”

Section: Discussionmentioning

confidence: 99%

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Incorporating circulating cytokines into the idiopathic inflammatory myopathy subclassification toolkit

Paepe

2023

Front. Med.

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Extensive diagnostic delays and deferred treatment impact the quality of life of patients suffering from an idiopathic inflammatory myopathy. In-depth subtyping of patients is a necessary effort to engage appropriate disease management and may require specialized and elaborate evaluation of the complex spectrum of clinical and pathological disease features. Blood samples are routinely taken for diagnostic purposes, with creatine kinase measurement and autoantibody typing representing standard diagnostic tools in the clinical setting. However, for many patients the diagnostic odyssey includes the invasive and time-consuming procedure of taking a muscle biopsy. It is proposed that further implementation of blood-based disease biomarkers represents a convenient alternative approach with the potential to reduce the need for diagnostic muscle biopsies substantially. Quantification of judicious combinations of circulating cytokines could be added to the diagnostic flowchart, and growth differentiation factor 15 and C-X-C motif chemokine ligand 10 come forward as particularly good candidates. These biomarkers can offer complementary information for diagnosis indicative of disease severity, therapeutic response and prognosis.

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Section: Discussionmentioning

confidence: 99%

“…An example are DM patients with characteristic muscle complaints and the hallmark skin lesions. However, DM patients may also present with muscle weakness only (DM sine dermatitis) or solely exhibit cutaneous manifestations (amyopathic DM) (38) signs with serological, pathological and genetic analyses. As much as a skilled neuropathologist is essential for the diagnosis of IIM, such an expert may be a rare find.…”

Section: Discussionmentioning

confidence: 99%

Incorporating circulating cytokines into the idiopathic inflammatory myopathy subclassification toolkit

Paepe

2023

Front. Med.

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“…It affects women more often than men. It mainly affects young adults, and juvenile-onset cases have been reported in the literature [ 6 ]. ADM is characterized by cutaneous lesions highly suggestive of dermatomyositis without clinical, biological, or histological muscle involvement (normal electromyogram, muscle enzymes, and muscle biopsy) [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Rapidly Progressive Interstitial Lung Disease With Pneumothorax and Pneumomediastinum Secondary to Amyopathic Dermatomyositis

Imzil,

Assoufi,

Mouline

et al. 2023

Cureus

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Amyopathic dermatomyositis is a rare form of dermatomyositis characterized by cutaneous lesions without clinical, biological, or histological muscular involvement. Pulmonary complications associated with this condition are diffuse interstitial lung disease (ILD), pneumomediastinum, and spontaneous pneumothorax. The form associated with anti-melanoma differentiation-associated protein 5 (anti-MDA-5) antibodies is reputed to have a poor prognosis and is responsible for ILD which can rapidly progress to fatal respiratory failure. Treatment of amyopathic dermatomyositis is essentially based on corticosteroid therapy and immunosuppressants. We present the case of a 42-year-old patient followed for three months for diffuse ILD. The patient was hospitalized for respiratory distress secondary to an aggravation of ILD with pneumomediastinum and pneumothorax. The patient also presented with cutaneous lesions identified as Gottron's papules. The patient was diagnosed with amyopathic dermatomyositis positive for anti-MDA-5 antibodies.

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“…2 However, a subtype known as amyopathic DM, which includes anti-MDA5 DM, usually presents without muscle involvement. 3 Clinical muscle weakness has been reported in cases of anti-MDA5 DM, though it is less likely in these patients. 4 The characteristic cutaneous phenotype of anti-MDA5 DM was described by Fiorentino et al 5 in 2011 through a seminal retrospective study.…”

Section: Commentmentioning

confidence: 96%

Ulcerative Heliotrope Rash in Antimelanoma Differentiation–Associated Gene 5 Dermatomyositis

Daugherty

Cheeley

2021

Cutis

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Antimelanoma differentiation-associated gene 5 dermatomyositis (anti-MDA5 DM) is an amyopathic subtype of DM that presents with the classic cutaneous findings of DM, such as a heliotrope rash, Gottron papules, and the shawl sign, combined with mucocutaneous ulcerations. This subtype of DM also is highly associated with rapidly progressive interstitial lung disease (ILD). We report 2 cases of a heretofore novel presentation of the overlap of these cutaneous features in the form of an ulcerative heliotrope rash; furthermore, the rash was associated with progression of ILD in both cases. Finally, we review the current state of clinical care and research related to anti-MDA5 DM in terms of clinical presentations, diagnostic methods, and treatment options.

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Amyopathic Dermatomyositis

Cited by 56 publications

References 23 publications

Incorporating circulating cytokines into the idiopathic inflammatory myopathy subclassification toolkit

Incorporating circulating cytokines into the idiopathic inflammatory myopathy subclassification toolkit

Rapidly Progressive Interstitial Lung Disease With Pneumothorax and Pneumomediastinum Secondary to Amyopathic Dermatomyositis

Ulcerative Heliotrope Rash in Antimelanoma Differentiation–Associated Gene 5 Dermatomyositis

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