Gottron Papules and Gottron Sign with Ulceration: A Distinctive Cutaneous Feature in a Subset of Patients with Classic Dermatomyositis and Clinically Amyopathic Dermatomyositis.
Gottron papules and Gottron sign are characteristic and possibly pathognomonic cutaneous features of classic dermatomyositis and clinically amyopathic dermatomyositis (DM/CADM). However, the Gottron papules/Gottron sign with cutaneous ulceration (ulcerative Gottron papules/Gottron sign) are less common. We aimed to clarify the clinical characteristics of patients with DM/CADM who have ulcerative Gottron papules/Gottron sign.



Clinical features, laboratory findings, and prognosis of patients with DM/CADM who had Gottron papules/Gottron sign with or without ulceration were analyzed and compared.



Occurrences of acute interstitial pneumonia/subacute interstitial pneumonia (AIP/SIP) were significantly higher in patients with ulcerative Gottron papules/Gottron sign (19/26) versus patients with Gottron papules/Gottron sign without ulceration (2/66, p < 0.001). We also observed that the white blood cell counts (mean ± SD 4.2 ± 1.6 vs 6.9 ± 2.9; p < 0.001) and creatine kinase (CK) levels (198.0 ± 377.7 vs 1364.0 ± 2477.0; p = 0.019) were significantly lower, whereas the positive rate of antimelanoma differentiation-associated gene 5 antibody (anti-MDA5; 88.5% vs 6.1%, p < 0.001) and serum ferritin levels (665.2 ± 433.5 vs 256.2 ± 279.0, p < 0.001) were significantly higher in the patients with ulcerative Gottron papules/Gottron sign. Moreover, the cumulative survival rate of the group with ulcerative Gottron papules/Gottron sign was significantly lower (p < 0.001).



Patients with DM/CADM who have ulcerative Gottron papules/Gottron sign, positive anti-MDA5 antibody, and significantly lower baseline CK level are at increased risk of interstitial lung disease, especially AIP/SIP. A new designation for this subgroup of patients should be established to draw more attention to this clinical entity.
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Hua
H
Cao
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Qunli
Q
Xia
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Meng
M
Pan
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Xiaoqing
X
Zhao
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Xia
X
Li
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Ruofei
R
Shi
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Min
M
Zhou
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Xiaoyi
X
Ding
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University.
Masataka
M
Kuwana
From the Department of Dermatology, the Department of Respiratory, and the Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China; the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.H. Cao, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; Q. Xia, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Pan, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Zhao, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Li, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; R. Shi, MD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Zhou, MD, PhD, Department of Respiratory, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; X. Ding, MD, PhD, Department of Radiology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University; M. Kuwana, MD, PhD, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; J. Zheng, MD, PhD, Department of Dermatology, Rui Jin Hospital, School of Medicine, Shanghai Jiao Tong University. jie-zheng2001@126.com.
Jie
J
Zheng
D000328:Adult; D003402:Creatine Kinase / Q000097:blood; D003882:Dermatomyositis / Q000097:blood / Q000150:complications / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007958:Leukocyte Count; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012867:Skin / Q000473:pathology*; D012883:Skin Ulcer / Q000097:blood / Q000150:complications / Q000473:pathology*
D003402:Creatine Kinase
AMYOPATHIC DERMATOMYOSITIS; DERMATOMYOSITIS; INTERSTITIAL LUNG DISEASE; SKIN ULCER