Fever, Inflammatory Response, and a Persistent Rash

Mediterranean Journal of Rheumatology

article

Dimitrios Daoussis¹  Pantelis Kraniotis²  Nikolaos Maltezos³ 

[1] Department of Rheumatology, University of Patras Medical School, Patras University Hospital;Department of Radiology, University of Patras Medical School, Patras University Hospital;Dermatologist

关键词: autoinflammatory diseases;    anakinra;    Schnitzler syndrome;    IL-1;   

DOI  :  10.31138/mjr.33.3.368

学科分类：社会科学、人文和艺术（综合）

来源: PCO Convin S.A.

【 摘 要 】

A 68-year-old Caucasian male was referred to theRheumatology Department with the clinical suspicion ofadult-onset Still’s disease (AOSD) since the patient hadfever, high inflammatory markers, a rash and an extensivework up had excluded infectious or neoplastic causes.Twelve months ago, the patient first developed a non-pruritic rash on his torso and arms that recurred frequently.During the last few months, the patient developed feverand significant weight loss, therefore a series of investigations was performed. Lab tests revealed an ESR of130mm/h, a 15-fold elevation of CRP, leucocytosis withnormal complement levels, and no autoantibodies. A fullbody CT and temporal artery biopsy were unremarkable.An IgMκ monoclonal gammopathy was found and thepatient was admitted to hospital for further investigationswith the initial suspicion being a hematologic malignancy.An extensive evaluation including bone marrow biopsyand PET/CT ruled out lymphomas, myeloproliferativedisorders or plasma cell dyscrasias whereas a thoroughwork up for infectious diseases was negative. Whenwe first evaluated the patient, we noticed that the rashhad urticaria-like features and was not compatible withAOSD (Figure 1A).

【 授权许可】

CC BY-NC