Anti-SAE1 Antibodies and Dermatomyositis: A Case Series of Three Patients

Claire Wilson,Brandon Roman,Iris K Aronson,Kurt Ashack

doi:10.25251/skin.4.6.15

Claire Wilson, Brandon Roman + Show 2 more

Open Access

https://doi.org/10.25251/skin.4.6.15

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Abstract

To date, few patients with anti-SAE1 dermatomyositis (DM) have been described, and usually in large descriptive cohort studies including all DM subtypes. Thus, it is increasingly important to further describe this rare subtype to solidify cutaneous findings, associated symptoms, and potential therapeutic options. Herein, this case series describes three patients with anti-SAE1 DM with respect to their clinical and laboratory findings, and also their response to treatment. All three patients eventually presented with classic cutaneous manifestations of dermatomyositis, however, Case 2 presented initially with cutaneous manifestations not described in the literature. Furthermore, two patients presented with dysphagia, with Case 2 experiencing a life-threatening dysphagia not typically described in the literature. Two of three patients also presented with early signs of interstitial lung disease. Lastly, cutaneous disease was only responsive to immunosuppressants in one patient. Unfortunately, this observational case-series consisted of only three patients, limiting the generalizability of our results. However, this study provides further support of the recalcitrant nature of this DM subtype, and commonly affected extracutaneous systems including the gastrointestinal and pulmonary systems. Larger studies of this patient population are needed to further confirm our associated findings.

Highlights

Diagnosing clinically amyopathic dermatomyositis (CADM) is a challenge for clinicians due to the lack of classic muscle findings associated with dermatomyositis (DM) and the wide array of clinical presentations depending on the associated antibody subtype.[1]
This subtype of CADM is often recalcitrant to treatment and may be associated with severe cutaneous disease, dysphagia, and interstitial lung disease.[3]
Over the several months, patient developed muscle weakness and dysphagia, which responded to a combination of monthly intravenous immune globulin (IVIg), mycophenolate mofetil (MMF), and prednisone

Summary

INTRODUCTION

Diagnosing clinically amyopathic dermatomyositis (CADM) is a challenge for clinicians due to the lack of classic muscle findings associated with dermatomyositis (DM) and the wide array of clinical presentations depending on the associated antibody subtype.[1]. Over the several months, patient developed muscle weakness and dysphagia, which responded to a combination of monthly intravenous immune globulin (IVIg), mycophenolate mofetil (MMF), and prednisone Her cutaneous disease did not improve with the above treatments in addition or to intramuscular and topical corticosteroids and worsened with hydroxychloroquine, which was discontinued. Hydroxychloroquine resolved her eroded DLE-like lesions, with multiple areas of scarring left behind Three months later, she developed extensive violaceous, erythematous, and edematous macules, papules, patches, and plaques on the face, neck, chest, arms, dorsal hands, and lateral thighs consistent with a diagnosis of dermatomyositis. She developed extensive violaceous, erythematous, and edematous macules, papules, patches, and plaques on the face, neck, chest, arms, dorsal hands, and lateral thighs consistent with a diagnosis of dermatomyositis She was subsequently hospitalized due to severe dysphagia, which improved with intravenous methylprednisolone.

DISCUSSION

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CONCLUSION