The Turkish Journal of Gastroenterology

To the Editor,
A 38-year-old man was hospitalized because of elevated serum liver enzyme levels, hepatomegaly, fever, bilateral lymphadenopathies on cervical chain, and extensive bullous skin lesions on his trunk, face and extremities (Figure 1). Symptoms appeared four weeks after the initial dose of sulfasalazine for rheumatoid arthritis. The medication was stopped. He had no alcohol history. Initial laboratory tests were as follows: white blood cell count (WBC) 19.3 (NR: 4.3–10.3x 103/μL), aspartate transaminase (AST) 127 U/L (NR: 10-40), alanine aminotransferase (ALT) 192 U/L (NR: 10-40), total bilirubin 3.3 mg/dl (NR: 0.2-1), alkaline phosphatase (ALP) 562 U/L (NR: 38-155), lactate dehydrogenase (LDH) 1118 U/L (NR: 220-450), and albumin 3.5 g/dl (NR: 3.5-5.5). All test results for viral and autoimmune hepatitis were negative. Serum anti-human herpes virus-6 (HHV-6) titer was interpreted as a weak level (Pasteur Cerba Laboratory, France). Serum Ig E level was remarkably high (>5000 kU/L). Blood film revealed 9% eosinophilia (NR: <6%) and 57% lymphocythemia (NR: 19.4-44.9%).
The Doppler ultrasonographic examination of the liver confirmed the enlargement with cranio–caudal length of 182 mm. Sonographic findings of liver vascular flows were normal.
Lymph node and skin punch biopsies were performed and histopathologic diagnoses were consistent with drug reaction. Surprisingly, pulmonary involvement developed, which was confirmed with laboratory and computed tomography (CT) scan during the hospitalization period, for which antibiotics were started.
Pulmonary, hepatic, skin, and lymph node involvements, hematological abnormalities like eosinophilia and lymphocythemia, marked elevation of Ig E, exclusion of other reasons for hepatitis, history of sulfasalazine treatment, and histopathologic examination results suggested sulfasalazine- induced DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) as described by Bocquet et al. (1).
On day 5 of hospitalization, a tapering dose of methyl prednisolone 60 mg was prescribed over five weeks. The initiation of corticosteroid therapy led to an immediate symptomatic improvement. Three months later, complete recovery was observed. DRESS syndrome carries an approximately 10% mortality rate (1-3) and in quite a few articles, mortality is reported to associate with HHV-6, which is a "harmless" virus in normal individuals (4). Although there was no sign of HHV-6 activation in our patient during or after corticosteroid therapy, in cases with high serum titers, it might be mortal under immunosuppressive drugs. Therefore, screening for HHV-6 in patients with DRESS syndrome before administration of immunosuppressive drugs should always be on the agenda.