CC BY
3mediterranean journal
of RHEUMATOLOGY
33 3
2022
:is D, Kraniotis P, Maltezos N.
This work is licensed under a Creative Commons Attribution 4.0 International L
CLINICAL IMAGE
Fever, Inflammatory Response, and a Persistent Rash
Dimitrios Daoussis1, Pantelis Kraniotis2, Nikolaos Maltezos3
department of Rheumatology, University of Patras Medical School, Patras University Hospital, Patras, Greece, department of Radiology, University of Patras Medical School, Patras University Hospital, 3Dermatologist, Patras, Greece
Mediterr J Rheumatol 2022;33(3):368 https://doi.org/10.31138/mjr.33.3.368
Article Submitted: 4 Mar 2022; Article Accepted: 30 Mar 2022; Available Online: 30 Sep 2022
Keywords: autoinflammatory diseases, anakinra, Schnitzler syndrome, IL-1
A 68-year-old Caucasian male was referred to the Rheumatology Department with the clinical suspicion of adult-onset Still's disease (AOSD) since the patient had fever, high inflammatory markers, a rash and an extensive work up had excluded infectious or neoplastic causes. Twelve months ago, the patient first developed a non-pru-ritic rash on his torso and arms that recurred frequently. During the last few months, the patient developed fever and significant weight loss, therefore a series of investigations was performed. Lab tests revealed an ESR of 130mm/h, a 15-fold elevation of CRP, leucocytosis with normal complement levels, and no autoantibodies. A full body CT and temporal artery biopsy were unremarkable. An IgMK monoclonal gammopathy was found and the patient was admitted to hospital for further investigations with the initial suspicion being a hematologic malignancy. An extensive evaluation including bone marrow biopsy and PET/CT ruled out lymphomas, myeloproliferative disorders or plasma cell dyscrasias whereas a thorough work up for infectious diseases was negative. When we first evaluated the patient, we noticed that the rash had urticaria-like features and was not compatible with AOSD (Figure 1A). Ferritin levels were within normal limits despite the robust inflammatory response making the diagnosis of AOSD even more unlikely. Since the rash was the first sign of the disease, a full review of the
patient's record was
Corresponding Author:
Dimitrios Daoussis
Department of Internal Medicine, Division
of Rheumatology
Patras University Hospital
26504 Rion, Patras, Greece
Tel.: +30 2613 603 693
Fax: +30 2610 993 982
E-mail: jimdaoussis@hotmail.com
performed in collaboration with dermatology colleagues. The combination of an urticarial rash, IgMK monoclonal gammopathy, fever and inflammatory response pointed towards Schnitzler's
Figure 1. (a) Skin rash with urticaria-like features. (b) Reformatted coronal oblique CT image (bone windows) at the level of the sacroiliac joints. Centred at the left ilium, there is a predominantly sclerotic lesion with lacelike pattern, narrow zone of transition and relatively well-defined margins (arrows). Note the presence of a similar smaller lesion on the right iliac bone (arrowheads).
syndrome, a rare but potentially underdiagnosed, au-toinflammatory disease of unknown aetiology. Notably, extensive osteosclerotic lesions in the pelvis were noted on imaging, also suggestive of the disease (Figure 1B). The patient started treatment with anakinra and exhibited a dramatic response with disappearance of skin lesions, and normalization of inflammatory markers.
CONFLICT OF INTEREST
The authors declare no conflict of interest.
FUNDING
This work was not funded by any source.
368 Cite this article as: Daoussis D, Kraniotis P, Maltezos N. Fever, Inflammatory Response, and a Persistent Rash. Mediterr J Rheumatol 2022;33(3):368.
