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Case report
Published online: 2025-02-12
A rare case of DRESS/DIHS with agranulocytosis progressing to MAS in a patient with psoriatic arthritis treated with sulfasalazine
Abstract
Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug-induced hypersensitivity syndrome (DIHS), is a rare hypersensitivity reaction usually presenting with eosinophilia, morbilliform rash, fever, lymphadenopathy, and internal organ involvement.
We present a rare case of DRESS/DIHS in a patient with psoriatic arthritis treated with sulfasalazine and meloxicam who developed agranulocytosis, confluent macular rash, fever, lymphadenopathy, and liver failure, progressing to macrophage activation syndrome (MAS) — a form of hemophagocytic lymphohistiocytosis (HLH).
Keywords: DRESSDIHSagranulocytosisneutropeniaMASHLHsulfasalazine
References
- Adwan MH. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome and the Rheumatologist. Curr Rheumatol Rep. 2017; 19(1): 3.
- Kardaun SH, Sekula P, Valeyrie-Allanore L, et al. RegiSCAR study group. Drug reaction with eosinophilia and systemic symptoms (DRESS): an original multisystem adverse drug reaction. Results from the prospective RegiSCAR study. Br J Dermatol. 2013; 169(5): 1071–1080.
- Radovanovic M, Jevtic D, Calvin AD, et al. "Heart in DRESS": Cardiac Manifestations, Treatment and Outcome of Patients with Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome: A Systematic Review. J Clin Med. 2022; 11(3).
- Taweesedt PT, Nordstrom CW, Stoeckel J, et al. Pulmonary Manifestations of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome: A Systematic Review. Biomed Res Int. 2019; 2019: 7863815.
- Jevtic D, Dumic I, Nordin T, et al. Less Known Gastrointestinal Manifestations of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome: A Systematic Review of the Literature. J Clin Med. 2021; 10(18).
- Naranjo CA, Busto U, Sellers EM, et al. A method for estimating the probability of adverse drug reactions. Clin Pharmacol Ther. 1981; 30(2): 239–245.
- Chen L, Chia C, Saha A. Drug Reaction with Eosinophilia and Systemic Symptoms and Agranulocytosis Presenting as Cervical Lymphadenopathy. Hosp Pharm. 2022; 57(1): 130–134.
- Fathallah N, Slim R, Rached S, et al. Sulfasalazine-induced DRESS and severe agranulocytosis successfully treated by granulocyte colony-stimulating factor. Int J Clin Pharm. 2015; 37(4): 563–565.
- Kato M, Kano Y, Sato Y, et al. Severe Agranulocytosis in Two Patients with Drug-induced Hypersensitivity Syndrome/Drug Reaction with Eosinophilia and Systemic Symptoms. Acta Derm Venereol. 2016; 96(6): 842–843.
- Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007; 48(2): 124–131.
- Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014; 66(9): 2613–2620.
- Ammar H, Azouzi A, Fathallah N, et al. Fatal sulfasalazine-induced DRESS complicated by HHV-6 reactivation and hemophagocytic lymphohistiocytosis. Eur J Clin Pharmacol. 2020; 76(3): 467–468.
- Komatsuda A, Okamoto Y, Hatakeyama T, et al. Sulfasalazine-induced hypersensitivity syndrome and hemophagocytic syndrome associated with reactivation of Epstein-Barr virus. Clin Rheumatol. 2008; 27(3): 395–397.
- Liang J, Qu H, Wang X, et al. Drug Reaction with Eosinophilia and Systemic Symptoms Associated with Reactivation of Epstein-Barr Virus and/or Cytomegalovirus Leading to Hemophagocytic Syndrome in One of Two Patients. Ann Dermatol. 2018; 30(1): 71–74.
- Yang JJ, Lei DK, Ravi V, et al. Overlap between hemophagocytic lymphohistiocytosis and drug reaction and eosinophilia with systemic symptoms: a review. Int J Dermatol. 2020 [Epub ahead of print].
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