Relapse of Juvenile Dermatomyositis following Eight Years in Remission: A Case Report.

Abstract

Introduction: Dermatomyositis is an autoimmune inflammatory condition that is characterized by proximal muscle weakness and a variety of cutaneous manifestations.

Case presentation: In this report, we discuss the case of a 27-year-old white female who developed a relapse of juvenile dermatomyositis (JDM) 8 years after remission. The patient presented with a 4-month history of progressive proximal muscle weakness and rash, as well as a more recent onset of dysphagia. Severe cutaneous involvement was apparent on examination with ulcerated, pruritic plaques, violaceous papules, and diffuse erythema. Laboratories revealed elevated CK, aldolase, and CRP, with positive anti-NXP2, anti-U1 RNP, and anti-U2 RNP antibodies. Based on the clinical picture and diagnostic results, a relapse of JDM was diagnosed and various medication trials were initiated. Since initial presentation, the patient has improved and is currently stable on a regimen of hydroxychloroquine, IVIG, low-dose corticosteroids, tofacitinib, and gabapentin. Her myositis is in remission; however, she continues to experience burdensome cutaneous symptoms and overall significant disease impact.

Conclusion: This case highlights the possibility of JDM relapse even after prolonged remission, underscoring the importance of maintaining a high index of suspicion for symptom recurrence. It also offers a thorough example of multimodal therapy use for refractory JDM.