Korean J Dermatol.  2024 Jan;62(1):42-45.

Adult-Onset Still’s Disease with Atypical Persistent Rash and Histologic Findings of Neutrophilic Urticarial Dermatosis

• Park YJ
• Shin HY
• Choi WK
• Sim HB
• Hong JS
• Lee AY
• Lee SH

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease characterized by spiking fever, arthralgia, skin rashes, and hyperferritinemia. The rash is usually salmon-colored, non-itchy, accompanied by fever, and disappears with an improvement of fever. However, in some cases, the rash persisted regardless of fever. Here, we present a case of AOSD with an atypical persistent rash that showed histological findings resembling those of neutrophilic urticarial dermatosis. The patient was a 60-year-old woman with high fever, arthralgia, and a persistent flagellated skin rash. Despite systemic steroid treatment, the patient developed a serious complication: macrophage activation syndrome. Since this case presented with an atypical persistent rash with histological resemblance to neutrophilic urticarial dermatosis, we report its contribution to the further study of AOSD.

Keyword

Adult-onset Still’s disease; Neutrophilic urticarial dermatosis