Late Onset Evanescent Rash in a Patient with Adult-Onset Still’s Disease

Abstract

Adult-onset Still’s disease is a rare multisystem autoimmune disease characterized by fever, skin rash, arthritis, and hyperferritinemia. Skin rash usually develops along with the peak of fever. Specific diagnostic test has not been established yet. Therefore, the diagnosis is usually based on symptom complex and the skin rash. We present a case of adult-onset Still’s disease in a healthy middle-aged man with prolonged fever for 4 month without any rash. The biopsy was taken from normal skin to exclude intravascular lymphoma. The evanescent rash developed 3 months after he had fever which raised physician about the diagnosis of adult-onset Still’s disease. According to Yamaguchi criteria, the diagnosis criteria in this patient composed of fever, sore throat, rash, leukocytosis and transaminitis. Late manifestation of skin rash was underrecognized which leaded to delay diagnosis. The early recognition could be leaded to early diagnosis and appropriate treatment. This report demonstrates a late onset of cutaneous manifestation in patient with adult-onset Still’s disease.