Abstract/Text: Dermatomyositis (DM) is a common idiopathic inflammatory myopathy. The pathogenesis is considered to be microangiopathy affecting skin and muscle. The cutaneous manifestations of DM are the most important aspect of this disease, and their correct evaluation is important for early diagnosis. The skin signs are various: Some are pathognomonic or highly characteristic, and others are compatible with DM. Recently, DM has been categorized into several disease subsets based on the various autoantibodies present in patients. Sometimes, characteristic cutaneous manifestations are strongly associated with the presence of specific autoantibodies. For example, anti-Mi-2 antibody is associated with the classic features of DM, including heliotrope rash, Gottron's papules, the V-neck sign, the shawl sign, cuticular overgrowth, and photosensitivity. Frequent cutaneous features in anti-transcriptional intermediary factor 1 gamma (TIF1γ)-positive patients are diffuse photoerythema, including "dusky red face," while skin ulcerations, palmar papules (inverse Gottron), diffuse hair loss, panniculitis, and oral pain and/or ulcers are sometimes associated with anti-melanoma differentiation-associated gene 5 product (MDA5) antibody. Here, we review important cutaneous manifestations seen in patients with DM, and we examine the relationship between the skin changes and myositis-associated autoantibodies. Correct evaluation of cutaneous manifestations and myositis-associated autoantibodies should help the clinician in the early diagnosis of DM, for a quick recognition of cutaneous signs that may be the symptom of onset before muscle inflammation.

Abstract/Text: Dermatomyositis (DM) is a common idiopathic inflammatory myopathy. The pathogenesis is considered to be microangiopathy affecting skin and muscle. The cutaneous manifestations of DM are the most important aspect of this disease, and their correct evaluation is important for early diagnosis. The skin signs are various: Some are pathognomonic or highly characteristic, and others are compatible with DM. Recently, DM has been categorized into several disease subsets based on the various autoantibodies present in patients. Sometimes, characteristic cutaneous manifestations are strongly associated with the presence of specific autoantibodies. For example, anti-Mi-2 antibody is associated with the classic features of DM, including heliotrope rash, Gottron's papules, the V-neck sign, the shawl sign, cuticular overgrowth, and photosensitivity. Frequent cutaneous features in anti-transcriptional intermediary factor 1 gamma (TIF1γ)-positive patients are diffuse photoerythema, including "dusky red face," while skin ulcerations, palmar papules (inverse Gottron), diffuse hair loss, panniculitis, and oral pain and/or ulcers are sometimes associated with anti-melanoma differentiation-associated gene 5 product (MDA5) antibody. Here, we review important cutaneous manifestations seen in patients with DM, and we examine the relationship between the skin changes and myositis-associated autoantibodies. Correct evaluation of cutaneous manifestations and myositis-associated autoantibodies should help the clinician in the early diagnosis of DM, for a quick recognition of cutaneous signs that may be the symptom of onset before muscle inflammation.

Abstract/Text: Dermatomyositis (DM) is a common idiopathic inflammatory myopathy. The pathogenesis is considered to be microangiopathy affecting skin and muscle. The cutaneous manifestations of DM are the most important aspect of this disease, and their correct evaluation is important for early diagnosis. The skin signs are various: Some are pathognomonic or highly characteristic, and others are compatible with DM. Recently, DM has been categorized into several disease subsets based on the various autoantibodies present in patients. Sometimes, characteristic cutaneous manifestations are strongly associated with the presence of specific autoantibodies. For example, anti-Mi-2 antibody is associated with the classic features of DM, including heliotrope rash, Gottron's papules, the V-neck sign, the shawl sign, cuticular overgrowth, and photosensitivity. Frequent cutaneous features in anti-transcriptional intermediary factor 1 gamma (TIF1γ)-positive patients are diffuse photoerythema, including "dusky red face," while skin ulcerations, palmar papules (inverse Gottron), diffuse hair loss, panniculitis, and oral pain and/or ulcers are sometimes associated with anti-melanoma differentiation-associated gene 5 product (MDA5) antibody. Here, we review important cutaneous manifestations seen in patients with DM, and we examine the relationship between the skin changes and myositis-associated autoantibodies. Correct evaluation of cutaneous manifestations and myositis-associated autoantibodies should help the clinician in the early diagnosis of DM, for a quick recognition of cutaneous signs that may be the symptom of onset before muscle inflammation.

Abstract/Text: BACKGROUND: Anti-CADM-140 antibody (anti-CADM-140), also referred to as anti-melanoma differentiation-associated gene 5 (MDA5) antibody, is a myositis-specific antibody identified in the sera of patients with clinically amyopathic dermatomyositis (C-ADM) and is associated with a worse prognosis in dermatomyositis-associated interstitial lung disease (DM-ILD). We sought to determine high-resolution computed tomography (HRCT) features of DM-ILD with anti-CADM-140.
METHODS: Twenty-five patients newly diagnosed with DM-ILD at Kyoto University Hospital between 2005 and 2009 were retrospectively reviewed. Serum anti-CADM-140 was measured in all patients at their first visit. Chest HRCT images taken prior to treatment were classified based on the dominant findings and their distribution, and compared between patients with and without the antibody.
RESULTS: Of 25 DM-ILD patients, 12 were positive and 13 were negative for anti-CADM-140. HRCT patterns differed significantly between anti-CADM-140-positive and negative patients (P = 0.002). Lower consolidation or ground-glass attenuation (GGA) pattern (50.0%) and random GGA pattern (33.3%) were the predominant patterns in anti-CADM-140-positive cases, while lower reticulation pattern (69.2%) was frequently seen in anti-CADM-140-negative cases. Anti-CADM-140-positive cases were also significantly characterized by the absence of intralobular reticular opacities (0% in anti-CADM-140 (+) vs. 84.6% in anti-CADM-140 (-), P < 0.0001).
CONCLUSIONS: Anti-CADM-140-positive DM-ILD was characterized by lower consolidation or GGA pattern, random GGA pattern, and the absence of intralobular reticular opacities.

Copyright © 2011 Elsevier Ltd. All rights reserved.

Abstract/Text: BACKGROUND: Anti-CADM-140 antibody (anti-CADM-140), also referred to as anti-melanoma differentiation-associated gene 5 (MDA5) antibody, is a myositis-specific antibody identified in the sera of patients with clinically amyopathic dermatomyositis (C-ADM) and is associated with a worse prognosis in dermatomyositis-associated interstitial lung disease (DM-ILD). We sought to determine high-resolution computed tomography (HRCT) features of DM-ILD with anti-CADM-140.
METHODS: Twenty-five patients newly diagnosed with DM-ILD at Kyoto University Hospital between 2005 and 2009 were retrospectively reviewed. Serum anti-CADM-140 was measured in all patients at their first visit. Chest HRCT images taken prior to treatment were classified based on the dominant findings and their distribution, and compared between patients with and without the antibody.
RESULTS: Of 25 DM-ILD patients, 12 were positive and 13 were negative for anti-CADM-140. HRCT patterns differed significantly between anti-CADM-140-positive and negative patients (P = 0.002). Lower consolidation or ground-glass attenuation (GGA) pattern (50.0%) and random GGA pattern (33.3%) were the predominant patterns in anti-CADM-140-positive cases, while lower reticulation pattern (69.2%) was frequently seen in anti-CADM-140-negative cases. Anti-CADM-140-positive cases were also significantly characterized by the absence of intralobular reticular opacities (0% in anti-CADM-140 (+) vs. 84.6% in anti-CADM-140 (-), P < 0.0001).
CONCLUSIONS: Anti-CADM-140-positive DM-ILD was characterized by lower consolidation or GGA pattern, random GGA pattern, and the absence of intralobular reticular opacities.

Copyright © 2011 Elsevier Ltd. All rights reserved.