Case Quiz (July 2016)
A 3-year-old girl who presented acute diarrhoea and icteric conjunctiva was given, successively, cotrimoxazole for 5 days and cefixime for 7 days, without any liver function test at this time. Three weeks later, she was admitted for a subfulminant hepatitis virus A infection, evidenced by high titres of specific antihepatitis virus A immunoglobulin (Ig)G and IgM. She had an ongoing fever, her prothrombin time was 27%, factor V level was 49%, AST and ALT levels were 160-times and 54 times,respectively, over the upper range of normal values. Electroencephalogram findings were normal. Strikingly, she developed eosinophilia 7 days after admission (1,000/μl),which rapidly increased to 30,000/μl, with simultaneous monocytosis (1,000/μl). There was no evidence of bacterial or parasitic infection, haematological malignancy or immunedeficiency. At this time, she did not receive any drugs other than vitamin supplementation. Non-oliguric acute renal failure (maximum serum creatinine level 3.4 mg/dl) occurred after 8 weeks of continuous fever and major eosinophilia. Renal histology displayed diffuse eosinophilic and lymphocyte interstitial infiltrate, with severe necrotic tubular lesions.There was moderate endocapillary proliferation, with mild glomerular eosinophilic infiltrate (see figures). Diagnosis of tubulointerstitial nephritis was made. No specific treatment was undertaken. Serum creatinine level decreased after 15 days (1.1 mg/dl) and was normal 1 month later (0.48 mg/dl). Hepatic biological parameters slowly improved. A mild eosinophilia persisted (2,700/μl)up to 2 months after the onset of renal failure and normalised subsequently. Diagnoses of infections with human herpes viruses 6 and 8, as well as Epstein–Barrvirus, were discarded as a result of the serology and polymerase chain reaction (PCR) findings.
Case Answer (July 2016)
DRESS is the acronym for Drug Reaction with Eosinophilia and Systemic Symptoms, an idiosyncratic, delayed, drug reaction occurring within 2 weeks to 6 weeks of the initiation of therapy. The hallmark of this syndrome is a protracted fever with multivisceral involvement (skin, liver,kidney) and major eosinophilia with monocytosis. Renal involvement is frequently reported. DRESS syndrome occurs frequently with concomitant viral infection, especially HHV6 and other Herpes viruses.
Acute renal failure, occurring in up to 30% of cases, is attributed to tubulointerstitial nephritis. Glomerular involvement has not been reported. Glomerular lesion reported in our case may be due to hepatitis virus A . Indeed,different renal lesions have been described with hepatitis virus A, including endocapillary proliferation.
Although DRESS syndrome is usually associated with herpes virus infection, our case suggests that hepatitis A virus infection may also be the only viral counterpart associated with the development of the DRESS syndrome.
