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

Ertao Jia,Hongling Geng,Jingjing Xie,Jianyong Zhang,Xia Qiu,Yuya Xiao,Yubao Jiang,Min Xiao,Yanying Zhang,Jiaxin Wei,Li Zhong

doi:10.1007/s10067-019-04562-w

Ertao Jia, Hongling Geng + Show 9 more

Open Access

https://doi.org/10.1007/s10067-019-04562-w

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Abstract

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).

Highlights

Anti-small ubiquitin-like modifier-1 activating enzyme antibodies have been recently discovered as myosin
A large-scale study on Chinese DM patients found that the frequency of anti-SAE antibody-positive DM was 3% [3]
A significantly higher frequency of anti-melanoma differentiation-associated gene 5 (MDA5) antibodies was observed in Chinese patients with polymyositis (PM)/DM than in Japanese patients [8]. These findings suggest that distinct genetic and/or local environmental factors affect Chinese and Japanese patients with PM/DM, who have been considered a homogeneous population in previous studies

Summary

Introduction

Anti-small ubiquitin-like modifier-1 activating enzyme (antiSAE) antibodies have been recently discovered as myosin-. A 48-year-old female presented diffuse pruritic erythema for 5 months. The erythema was distributed on her scalp, face, neck, chest, and back (Fig. 1a–c). Other skin manifestations included heliotrope rash, Gottron’s signs, V-neck sign, shawl. Changed to 100 mg of cyclosporine and 10 mg of prednisolone. The pruritic erythema improved after adding 50 mg of thalidomide daily for 6 months (Fig. 1d–f). The total activity score was 4, and total damage score was 0

Discussion

Findings

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