Association of juvenile dermatomyositis and juvenile lupus erythematosus: a case report

Abstract

Juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM) are two autoimmune diseases that can affect children and adolescents. JSLE is characterized by an autoimmune response that can affect multiple organs and systems, presenting with varied manifestations such as skin rashes, arthritis, hematologic manifestations, nephropathy, among others. On the other hand, JDM is an idiopathic inflammatory myopathy that is characterized by proximal muscle weakness and pathognomonic skin lesions, such as heliotrope and Gottron’s papules. The concomitant occurrence of these two autoimmune diseases in young patients is a rare but clinically relevant phenomenon that can impact diagnosis and treatment. This coexistence can lead to a more severe clinical case that is resistant to conventional therapies. CASE REPORT Female, 15 years old, student, was admitted to a hospital due to proximal weakness in the upper and lower limbs, persistent fever for a week and facial edema. Previously used high-dose prednisone, azathioprine, and hydroxychloroquine since the diagnosis of dermatomyositis. Onset of symptoms in December 2023 with proximal weakness, Gottron’s papules, and heliotrope. Due to pancytopenia, a bone marrow biopsy was performed, presenting globally hypocellular in the three hematopoietic series. In hospitalization exams, pancytopenia persisted, proteinuria was present and increased muscle enzymes were present. In the screening exams for lupus, anti-dsDNA and antinuclear antibody (ANA) were positive. After pulse therapy with methylprednisolone, the patient showed improvement in the clinical condition, currently using mycophenolate for maintenance therapy. CONCLUSION The overlap of JSLE and JDM presents a significant clinical challenge due to the complexity of symptoms and the need for a multifaceted therapeutic approach. Early and accurate identification of these diseases, together with an effective treatment strategy, is crucial to improve the prognosis and quality of life of affected patient