Clinical Images: Erythema on the toes in anti‐melanoma differentiation‐associated gene 5 antibody–positive dermatomyositis

The patient, a Japanese woman, was diagnosed with anti-melanoma differentiation-associated gene (MDA) 5 antibody–positive clinically amyopathic dermatomyositis (DM) complicated with interstitial lung disease (ILD) at the age of 63 years old. She was then treated with prednisolone (PSL), cyclosporine, and intravenous cyclophosphamide, which improved her symptoms, and she continued to receive oral PSL (3 mg/day) and cyclosporine (100 mg/day). (A–C) However, at the age of 70 years old, she developed erythema on her fingers, known as Gottron sign and inverse Gottron sign, along with new erythema on the dorsal and plantar surfaces of her toes. The arrowheard indicates the area of erythema. (D) Her anti-MDA5 antibody titer was elevated, and high-resolution chest computed tomography revealed reticular shadows and consolidations on the dorsal side of the bilateral lower lobes. These findings indicated a relapse of the disease, and treatment with increased doses of PSL and cyclosporine and readministration of intravenous cyclophosphamide promptly alleviated her symptoms. Her clinical course up to this point has been previously detailed in a case report.1 (E–H) Seven years later, she has maintained improvement in her skin and lung lesions without immunosuppressants. Because anti-MDA5 antibody–positive DM is often complicated by rapidly progressive ILD, especially in East Asia, including Japan, early identification of disease signs is crucial.2 The specific skin lesions of anti-MDA5 antibody–positive DM include the inverse Gottron sign and skin ulcers in addition to Gottron sign and heliotrope rash, which are classic manifestations of DM.3 Exacerbation of skin lesions often correlates with the exacerbation of ILD. Although lesions on the toes are rare in anti-MDA5 antibody–positive DM, their presence may lead to early detection of anti-MDA5 antibody–positive DM. Thus, observation of lesions extending to the toes is crucial. We would like to thank Editage (www.editage.jp) for English language editing. Disclosure form. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

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Journal
ACR Open Rheumatology
Published
2025-03-01
DOI
https://doi.org/10.1002/acr2.70011
Primary Topic
Inflammatory Myopathies and Dermatomyositis
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article
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Clinical Images: Erythema on the toes in anti‐melanoma differentiation‐associated gene 5 antibody–positive dermatomyositis

Yushiro Endo, Masataka Umeda, Toshimasa Shimizu, Atsushi Kawakami
ACR Open Rheumatology
Inflammatory Myopathies and Dermatomyositis
article

Clinical Images: Erythema on the toes in anti‐melanoma differentiation‐associated gene 5 antibody–positive dermatomyositis

Yushiro Endo, Masataka Umeda, Toshimasa Shimizu, Atsushi Kawakami
article en

Abstract

The patient, a Japanese woman, was diagnosed with anti-melanoma differentiation-associated gene (MDA) 5 antibody–positive clinically amyopathic dermatomyositis (DM) complicated with interstitial lung disease (ILD) at the age of 63 years old. She was then treated with prednisolone (PSL), cyclosporine, and intravenous cyclophosphamide, which improved her symptoms, and she continued to receive oral PSL (3 mg/day) and cyclosporine (100 mg/day). (A–C) However, at the age of 70 years old, she developed erythema on her fingers, known as Gottron sign and inverse Gottron sign, along with new erythema on the dorsal and plantar surfaces of her toes. The arrowheard indicates the area of erythema. (D) Her anti-MDA5 antibody titer was elevated, and high-resolution chest computed tomography revealed reticular shadows and consolidations on the dorsal side of the bilateral lower lobes. These findings indicated a relapse of the disease, and treatment with increased doses of PSL and cyclosporine and readministration of intravenous cyclophosphamide promptly alleviated her symptoms. Her clinical course up to this point has been previously detailed in a case report.1 (E–H) Seven years later, she has maintained improvement in her skin and lung lesions without immunosuppressants. Because anti-MDA5 antibody–positive DM is often complicated by rapidly progressive ILD, especially in East Asia, including Japan, early identification of disease signs is crucial.2 The specific skin lesions of anti-MDA5 antibody–positive DM include the inverse Gottron sign and skin ulcers in addition to Gottron sign and heliotrope rash, which are classic manifestations of DM.3 Exacerbation of skin lesions often correlates with the exacerbation of ILD. Although lesions on the toes are rare in anti-MDA5 antibody–positive DM, their presence may lead to early detection of anti-MDA5 antibody–positive DM. Thus, observation of lesions extending to the toes is crucial. We would like to thank Editage (www.editage.jp) for English language editing. Disclosure form. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

ACR Open RheumatologyVol. 7(3)
Nagasaki University Hospital (JP), Nagasaki University (JP)
Good health and well-being
Openalex Percentile: Top 93%
Inflammatory Myopathies and Dermatomyositis
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