A 13-year-old systemic lupus erythematosus girl initially presenting with Raccoon eyes and neuropsychiatric manifestations

Abstract

Background and aim of the workSystemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Although eyes are seldom affected, this case report presents a 13-year-old Iranian girl with Raccoon eyes and neuropsychiatric manifestations as an initial presentation of SLE. Case presentationThe patient was admitted to the pediatric rheumatology inpatient, Mofid Children’s Hospital, Tehran with swelling and ecchymosis around the eyes (Racoon eyes) as well as anorexia, abdominal pain, weight loss, mood disorders and hallucinations. Complete blood count showed normocytic, normochromic anemia, leukopenia, lymphopenia and thrombocytopenia. The C-reactive protein, erythrocyte sedimentation rate, liver and renal functional tests, and urine analysis were normal. The complement levels decreased, antinuclear antibody (ANA) and anti-double stranded DNA (anti-dsDNA) tests were positive. Bilateral pleural effusion and mild pericardial effusion were seen. Bone marrow aspiration showed mild hypocellularity without any evidence of malignancy. The diagnosis of neuropsychiatric SLE (NP-SLE) was held. She was treated by the pulse methyl prednisolone (30 mg/kg/d) and intravenous cyclophosphamide (500 mg/m2), oral prednisolone and hydroxychloroquine. Her appetite improved while hallucination and aggressive behavior decreased. Peri-orbital swelling and ecchymosis decreased. After one year, her appetite became normal; mood disorders, panic, phobic attacks and hallucinations were completely remedied. Swelling and ecchymosis around the eyes were eliminated. Oral prednisolone 10 mgday and hydroxychloroquine (5 mg/kg/day) were continued. ConclusionSLE may present with Raccoon eyes. Rapid detection and treatment of the disease based on clinical symptoms is critical for these patients. Prednisolone and cyclophosphamide are the best choice for treatment of the disease in children.