Refractory neuro-Sweet disease successfully treated with tocilizumab and mycophenolate mofetil

Sungeun Hwang; Hyoshin Son; Jangsup Moon; Soon-Tae Lee; Keun Hwa Jung; Kyung-Il Park; Sang Kun Lee; Kon Chu

doi:10.47936/encephalitis.2020.00059

Refractory neuro-Sweet disease successfully treated with tocilizumab and mycophenolate mofetil

Encephalitis. 2021 Jan;1(1):20-24. doi: 10.47936/encephalitis.2020.00059. Epub 2020 Dec 24.

Authors

Sungeun Hwang^{1

2}, Hyoshin Son^{1

2}, Jangsup Moon^{1

2

3}, Soon-Tae Lee^{1

2}, Keun Hwa Jung^{1

2}, Kyung-Il Park^{1

2

4}, Sang Kun Lee^{1

2}, Kon Chu^{1

2}

Affiliations

¹ Department of Neurology, Seoul National University Hospital, Seoul, Korea.
² Laboratory for Neurotherapeutics, Center for Medical Innovations, Biomedical Research Institute, Seoul National University Hospital, Seoul, Korea.
³ Rare Disease Center, Seoul National University Hospital, Seoul, Korea.
⁴ Department of Neurology, Seoul National University Hospital Healthcare System Gangnam Center, Seoul, Korea.

Abstract

Sweet syndrome, or acute febrile neutrophilic dermatosis, is mainly a dermatologic condition presenting with erythematous plaques; however, neutrophils infiltrate multiple systems. Neuro-Sweet disease is a neurological manifestation of Sweet syndrome and a rare cause of recurrent aseptic meningoencephalitis, which needs to be distinguished from neuro-Behçet disease. Although neuro-Sweet disease generally responds well to corticosteroids, relapsing neuro-Sweet disease is not an exceptional case. Herein, we present a case of a 51-year-old male with recurrent encephalitis followed by erythematous plaques. The patient was confirmed as Sweet syndrome based on skin biopsy and showed partial response to corticosteroids. With intravenous immunoglobulin, rituximab, tocilizumab, and mycophenolate mofetil, his neurologic symptoms were fully recovered.

Keywords: Mycophenolic acid; Sweet syndrome; Tocilizumab.

Publication types

Case Reports