A 57-year-old Japanese male visited to our department for high fever (> 38°C) lasting for 6 days with erythema multiforme-like rash on his extremities and trunk. He had pulmonary edema, and a laboratory test showed high CRP levels and elevated D-dimer. A blood culture was negative for facultative bacteria. Serological analysis with paired sera showed a slight elevation of IgG antibody titers specific for Japanese spotted fever (JSF). On the other hand, a PCR test of peripheral blood mononuclear cells was negative for JSF. The patient did not have any stab wound or skin rash typical of JSF. Since the test results were contradictory, we performed a next-generation sequencing analysis. As a result, specific genes for Rickettsia japonica were detected in peripheral blood mononuclear cells. Based on this result, we diagnosed the patient with Japanese spotted fever showing erythema multiforme-like eruption.
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.