Early-onset systemic lupus erythematosus with discoid rash and chondritis

Juvenile systemic lupus erythematosus (jSLE) is a rare disorder that presents with greater severity compared to adult systemic lupus erythematosus. It typically manifests in adolescent females with constitutional symptoms, multisystem involvement, and a malar rash. In this case, a 9-year-old African American pre-pubertal girl presented with a progressive facial and scalp rash, weight loss, night sweats without fever, and abdominal pain following recent penicillin exposure. On laboratory results and clinical examination, leucopenia, auricular chondritis, palatal ulceration, and marked facial rash were found on physical exam and initial lab testing, alongside a negative infectious disease workup. Autoimmune workup demonstrated an elevated antinuclear antibody and anti-double-stranded DNA with low complement levels. Therefore, she met diagnostic criteria for jSLE. She improved rapidly with corticosteroids. Over the following year, she subsequently developed lupus nephritis. This case illustrates an atypical presentation of jSLE with early-onset prior to puberty and uncommon features, including chondritis, discoid lesions, and isolated mucocutaneous symptoms, with no associated joint pain or organ damage at initial presentation. Atypical jSLE presentations require high clinical suspicion to prevent delayed diagnosis and progression to complications such as lupus nephritis.