Diffuse pruritic erythema as a clinical manifestation in anti-SAE antibody-associated dermatomyositis: a case report and literature review

• Published in: Clinical rheumatology 2019-08, Vol.38 (8), p.2189-2193
• Main Authors: Jia, Ertao, Wei, Jiaxin, Geng, Hongling, Qiu, Xia, Xie, Jingjing, Xiao, Yuya, Zhong, Li, Xiao, Min, Zhang, Yanying, Jiang, Yubao, Zhang, Jianyong
• Format: Article
• Language: English
• Subjects: Antibodies; Antibodies - immunology; Asian Continental Ancestry Group; Case Based Review; Case reports; Cyclosporine - administration & dosage; Cyclosporins; Dermatomyositis; Dermatomyositis - complications; Dermatomyositis - ethnology; Dysphagia; Erythema; Erythema - complications; Erythema - ethnology; Female; Humans; Immunoglobulins; Literature reviews; Lung diseases; Malignancy; Medicine; Medicine & Public Health; Methotrexate - administration & dosage; Middle Aged; Myosin; Patients; Prednisolone; Prednisolone - administration & dosage; Pruritus - complications; Pruritus - ethnology; Rheumatology; Thalidomide; Thalidomide - administration & dosage; Treatment Outcome; Ubiquitin; Ubiquitin-Activating Enzymes - immunology
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-019-04562-w

Anti-small ubiquitin-like modifier-1 activating enzyme (anti-SAE) antibodies have been recently discovered especially for myosin and identified as dermatomyositis (DM) marker. The frequency of anti-SAE antibodies in DM patients is extremely low. Diffuse pruritic erythema may be one kind of clinical manifestations of DM with anti-SAE antibodies. In this report, a 48-year-old female patient with amyopathic dermatomyositis (ADM) carrying anti-SAE antibodies presented diffuse pruritic erythema for 5 months. Diffuse pruritic erythema improved after treatment with prednisolone, cyclosporine, and thalidomide. The clinical characteristics of 75 previously reported cases with anti-SAE antibody-positive DM were reviewed, and the manifestations of the Asian and Western cohorts were compared. It was revealed that the Asian patients were more susceptible to diffuse erythema (17/34 vs. 3/41, P  = 0.000), dysphagia (16/34 vs. 10/41, P  = 0.040), and interstitial lung disease (ILD) (21/34 vs. 5/41, P  = 0.000) compared with the Western patients. The frequency of malignancy in the Asian cohort was significantly higher than that in the Western cohort (10/34 vs. 4/41, P  = 0.030).