Abstract:

Stills disease is an uncommon idiopathic inflammatory condition characterized by its cutaneous and systemic symptoms, including high fevers, polyarthralgia, and neutrophilia. The skin manifestations typically appear as evanescent, non-pruritic, salmon-colored morbilliform eruptions. However, a less common subtype has been described consisting of pruritic, persistent papules and plaques most commonly described in adults. We present a case of a 5 year-old girl initially seen by rheumatology with a pruritic, violaceous papular rash on her chest, back, hands, neck and cheeks over a month as well as two weeks of fever as high as 39C and one week of pain in her large joints, fingers, and back. The rash was more prominent when her fever spiked. Significant laboratory values included an elevated ESR and leukocytosis with neutrophilia. She was diagnosed with systemic Juvenile idiopathic arthritis (JIA) and started on immunosuppressants. On follow-up with dermatology, a punch biopsy performed on her back was notable for superficial epidermal dyskeratosis and a sparse perivascular chronic inflammatory infiltrate. Given the clinical presentation in the setting of JIA, she was diagnosed with persistent papules and plaques associated with Stills disease. As this is rarely reported in children, we wish to highlight this case to discuss the importance of clinicopathologic correlation in this setting.

Financial Disclosure:
No current or relevant financial relationships exist.

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Rebecca Czaja, MD