A RARE PRESENTATION OF SYSTEMIC-ONSET POLYARTHRITIC JUVENILE IDIOPATHIC ARTHRITIS WITH SEVERE ITCHING WITHOUT RASH

Abstract

Systemic-onset juvenile idiopathic arthritis (SJIA) is a distinct subtype of juvenile idiopathic arthritis characterized by quotidian fever, arthritis, lymphadenopathy, hepatosplenomegaly, and an evanescent rash. Dermatologic manifestations are typical in systemic disease, commonly presenting as a salmon-pink rash. However, isolated severe pruritus without rash is extremely uncommon and may create diagnostic uncertainty. We report a 5-year-old male with a 2-month history of intermittent high-grade fever, progressively worsening polyarthritis, and severe pruritus without visible skin rash. Examination revealed generalized lymphadenopathy, multiple polyarticular joint swellings, and scratch marks without erythema. Laboratory evaluation showed anemia, neutrophilic leukocytosis, thrombocytosis, markedly elevated C-reactive protein, and positive antinuclear antibody with nuclear and speckled pattern. Radiography revealed periarticular osteopenia without erosions. The child was treated with NSAIDs, antibiotics, and subsequently high-dose pulse methylprednisolone following ANA positivity, resulting in dramatic clinical improvement. He was discharged on weekly methotrexate with folic acid supplementation. This case highlights an atypical presentation of SJIA where severe itching without rash may obscure the diagnosis. Early recognition and initiation of immunosuppressive therapy can prevent long-term sequelae.