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

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 48year-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).

A North American Cohort of Anti‐SAE Dermatomyositis: Clinical Phenotype, Testing, and Review of Cases

“…As has been noted in other antibody specificities, there may be a difference in presentation depending on the ethnic background (18,19). A comparison of Eastern and Western case descriptions suggests that there is a higher risk for diffuse erythema, dysphagia, ILD, and malignancy in Eastern groups (14). The Johns Hopkins cohort used in this study more closely resembles Western cohorts given our predominantly Caucasian population.…”

A rare form of dermatomyositis associated with muscle weakness and normal creatine kinase level

“…Anti-SAE1-antibodies are also associated with CADM and skin features (80% of cases) with Gottron’s papules (64%), Gottron’s sign (64%), heliotrope rash (82%) and a distinct diffuse pruritic erythema, which is more common in Asians (50% of cases) than Caucasians (7.3%) 9 14 26. Anti-SAE1 antibodies are also associated with dysphagia (78%), higher rates of malignancy compared with other forms of DM (18.7% to 57% vs 9.4%) and less severe forms of ILD 9 15 26 27. There are no known reports of cardiomyopathy associated with anti-SAE1 antibodies.…”

The chameleon rash: a review of the polyphenotypic dermatoses of dermatomyositis

“…In the acute phase, the dermatosis is typically itchy and sore. Patients who carry the SAE antibody are especially prone to diffuse, pruritic erythema 22 . One study has reported SAE‐positive patients with a symmetrical area of sparing each side of the upper back, the so‐called ‘angel wings’ sign 23 (Fig.…”