Case report: Drug rash with eosinophilia and systemic symptoms syndrome in a patient with anti–interferon-γ autoantibody–associated immunodeficiency

• Published in: Frontiers in immunology Vol. 13; p. 969912
• Main Authors: Nie, Yuxue, Wang, Han, Dong, Xiying, Pan, Siqi, Zhang, Ting, Ran, Jun, Zhang, Ying, Fan, Junping, Zhang, Linqi, Wang, Jinglan
• Format: Journal Article
• Language: English
• Published: Frontiers Media S.A 22.08.2022
• Subjects: adult-onset immunodeficiency due to anti-interferon-gamma antibodies; anti-IFN-γ autoantibodies; Burkholderia; drug rash with eosinophilia and systemic symptoms; Immunology; infection; sulfamethoxazole
• ISSN: 1664-3224; 1664-3224
• DOI: 10.3389/fimmu.2022.969912

A 56-year-old Chinese woman with previous disseminated mycobacterium avium complex infection and recurrent cervical abscesses from Burkholderia cepacia complex visited our hospital. She was diagnosed with adult-onset immunodeficiency (AOID) and tested positive for interferon-γ–neutralizing autoantibody. Ceftazidime was administered as the initial antimicrobial treatment, which was later combined with sulfamethoxazole-trimethoprim (SMZ-TMP). She developed drug rash with eosinophilia and systemic symptoms (DRESS) syndrome after SMZ-TMP administration and improved after withdrawal of the culprit antibiotic and systemic glucocorticoids treatment. Her cervical infection was eventually cured after combined therapy of long-term antibiotics and anti–IFN-γ autoantibodies (AIGA) titer-lowering treatments including glucocorticoids, rituximab, and plasmapheresis. This is the first case of DRESS syndrome in the setting of AIGA-induced AOID and is worthy of notice.