伴有溃疡的Gottron丘疹和Gottron征：经典皮肌炎和临床无肌病性皮肌炎患者亚组中的一种独特皮肤表现。

Gottron Papules and Gottron Sign with Ulceration: A Distinctive Cutaneous Feature in a Subset of Patients with Classic Dermatomyositis and Clinically Amyopathic Dermatomyositis.

作者信息

Cao Hua, Xia Qunli, Pan Meng, Zhao Xiaoqing, Li Xia, Shi Ruofei, Zhou Min, Ding Xiaoyi, Kuwana Masataka, Zheng Jie

机构信息

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.

出版信息

J Rheumatol. 2016 Sep;43(9):1735-42. doi: 10.3899/jrheum.160024. Epub 2016 Jun 15.

DOI:10.3899/jrheum.160024

PMID:27307530

Abstract

OBJECTIVE

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.

METHODS

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.

RESULTS

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).

CONCLUSION

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.

摘要

目的

Gottron丘疹和Gottron征是典型皮肌炎和临床无肌病性皮肌炎（DM/CADM）的特征性且可能具有诊断特异性的皮肤表现。然而，伴有皮肤溃疡的Gottron丘疹/Gottron征（溃疡性Gottron丘疹/Gottron征）较少见。我们旨在阐明患有溃疡性Gottron丘疹/Gottron征的DM/CADM患者的临床特征。

方法

分析并比较有或无溃疡的Gottron丘疹/Gottron征的DM/CADM患者的临床特征、实验室检查结果及预后。

结果

溃疡性Gottron丘疹/Gottron征患者发生急性间质性肺炎/亚急性间质性肺炎（AIP/SIP）的比例（19/26）显著高于无溃疡的Gottron丘疹/Gottron征患者（2/66，p<0.001）。我们还观察到，溃疡性Gottron丘疹/Gottron征患者的白细胞计数（均值±标准差 4.2±1.6对6.9±2.9；p<0.001）和肌酸激酶（CK）水平（198.0±377.7对1364.0±2477.0；p = 0.019）显著更低，而抗黑色素瘤分化相关基因5抗体（抗MDA5）阳性率（88.5%对6.1%，p<0.001）和血清铁蛋白水平（665.2±433.5对256.2±279.0，p<0.001）显著更高。此外，溃疡性Gottron丘疹/Gottron征组的累积生存率显著更低（p<0.001）。