2023 Volume 133 Issue 3 Pages 481-491
The patient was a 33-year-old woman who had been treated with oral hydroxychloroquine sulfate and low-dose steroids for lupus erythematosus profundus. After the first COVID-19 vaccination dose, new subcutaneous nodules appeared on her arms, legs, and lower back. Three months after the second vaccination dose, she developed leg pain, remittent fever, erythema at the onset of fever, and hyperferritinemia. These symptoms met the diagnostic criteria for adult-onset Still's disease. The subcutaneous nodules showed marked mucin deposition and lobular panniculitis on histology. Computed tomography revealed extensive panniculitis in both her legs. A combination of steroid pulse therapy and cyclosporine resulted in improvement in her symptoms within two months. We speculate that the patient developed adult-onset Still's disease and macrophage activation syndrome after COVID-19 vaccination.
