Drug reaction with eosinophilia and systemic symptoms syndrome secondary to acetazolamide associated with markedly elevated procalcitonin

Abstract

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is an important cause of multi-organ dysfunction and can mimic other disorders including sepsis. We describe a patient presenting with septic shock and accompanying high procalcitonin. Although initially treated empirically with antibiotics, the emergence of eosinophilia during the admission lead to a revised diagnosis of DRESS syndrome, presumed secondary to acetazolamide. This case highlights the importance of regular clinical assessment and re-evaluation is key in identifying emerging features such as eosinophilia, rash and organ dysfunction, which can secure the diagnosis. Furthermore, the case also highlights that acetazolamide may be a rare cause of DRESS syndrome.

Keywords: adult intensive care; immunology; ophthalmology; unwanted effects / adverse reactions.

Figure 1

Drug timeline and days preceding hospital admission. Cataract procedures: right −25 days and left −12 days from admission. ***More than 6 months prior: multivitamin/mineral 10 g, D-aspartic acid 3 g, trimethyl glycine 500 mg, astaxanthine 4 mg, black cherry extract 750 mg, calcium reparen 750 mg, tinnitus formula, B-complex, phytosteral complex, pollen extract 500 mg, pygeum/saw palmetto, 5-hydroxytryptophan 100 mg, probiotic 250 mg, glucosamine sulphate 1000 mg, coenzyme Q10 100 mg, calamari oil 1000 mg, vitamin C 1000 mg, flaxseed oil 1000 mg, magnesium L-threonate 144 mg and gamma-aminobutyric acid. i.c., intra-cameral.

Figure 2

Drug timeline at presentation and days following admission. The presence of high-grade fever (>38°C), rash and facial swelling depicted. Intensive care unit management shown with haemodialysis and vasopressor support. Drugs prescribed on day 35 were continued through to discharge from hospital on day 46.

Figure 3

(A) Widespread erythematous fine pustular and maculopapular rash; histopathology from skin biopsy obtained from left chest wall. (B) Low power image showing suppurative folliculitis, characterised by neutrophils within the lumen and lining epithelium of the hair follicle. (C) High power image showing mixed dermal inflammatory infiltrate, including lymphocytes, histiocytes, neutrophils and eosinophils.

Figure 4

Blood results through hospital admission. (A) Liver enzymes ALT (range: 0–55 U/L), GGT (range: 0–60 U/L) and ALP (range: 30–110 U/L). (B) CRP (range: 0–8 mg/L), platelet count (range: 150×10⁹/L–450×10⁹/L) and creatinine (range: 45–90 μmol/L). (C) Eosinophil count (range: 0.02×10⁹/L–0.5×10⁹/L). ALP, alkaline phosphatase; ALT, alanine aminotransferase; CRP, C reactive protein; GGT, gamma glutamyl transferase.

Figure 5

Flow chart of differential diagnoses with progression of each clinical and or laboratory feature. ANCA, Anti-neutrophil cytoplasmic antibody, DDx, differential diagnosis; DRESS, drug reaction with eosinophilia and systemic symptoms; EBV, Epstein-Barr Virus; RegiSCAR, Registry of Severe Cutaneous Adverse Reactions to Drugs and Collections of Biological Samples; SJS, Stevens-Johnson Syndrome; TEN, toxic epidermal necrolysis.