Acute renal failure and febrile rash--infectious or not?

A previously healthy 45-year-old African-American man was admitted for acute renal failure. Recent history revealed cervical lymphadenopathy swelling, followed by apparition of fever and erythematous maculopapular rash on the abdomen and extremities. On admission, temperature was 38.6°C, blood pressure 119/79 mmHg. He had an erythematous rash on the arms and legs, with indurative oedema of the palms and soles, and bilateral non-suppurative conjunctivitis. Pharynx was injected. The patient also presented bilateral knee and ankle arthritis. Abdominal, cardiovascular and neurological examinations were normal. Laboratory studies showed: white cells count 15.9 × 109/l, neutrophils 13 × 109/l, eosinophils 1.1 × 109/l, CRP 275 mg/l, serum creatinine 215 µmol/l. SGOT, SGPT, total bilirubin, creatine kinase and lacticodeshydrogenase were normal, but γ-glutamyl transferase was 4 times the normal level and alkaline phosphatases 1.5 times the normal level. Urinalysis showed haematuria (80 cells/μl), leucocyturia (70 cells/μl), and only traces of proteinuria. ANCA, antinuclear antibodies, anti-dsDNA antibodies, rheumatoid factor and cryoglobulinaemia were negative. Complement was normal. Blood and urine bacterial cultures were negative. Serological tests were negative for Rickettsia, Coxiella, Chlamydia, Mycoplasma, Salmonella, Campylobacter jejuni, Bartonella, Leptospira, Hepatitis B, C, human immunodeficiency virus (HIV), parvovirus B19 and toxoplasmosis. There were cytomegalovirus and Epstein–Barr virus-specific IgG, with no IgM. Renal and abdominal ultrasonography showed two normal-sized kidneys, with mild hepatomegaly. Electrocardiogram, chest X-ray and two-dimensional echocardiogram were normal. What is your diagnosis? Patient's picture of the left hand. Note the peeling of the skin, beginning in the hyponychium, and the desquamation of the first and second fingers. He was treated with intravenous immunoglobulin. No aspirin therapy was given, because of an allergy. Temperature decreased and he gradually improved. Creatinine was 117 µmol/l the day of discharge, with normal urinalysis; CRP was 28mg/l. Two months after hospitalization, he had recovered normal renal function, and had not presented any complication. KD, also known as mucocutaneous lymph node syndrome, is a multisystemic vasculitis of the medium-sized vessels, most commonly diagnosed in young children (89% are under 5 years of age). The disease is characterized by high fever, rash, erythema and oedema of the extremities, lymphadenopathy, mucositis and non-purulent conjunctivitis. The main complication is the development of coronary aneurysms [1]. Clinical criteria for the diagnosis in children are established [1]. Treatment with intravenous immunoglobulin and aspirin has shown a significant reduction in the rate of cardiac aneurysms [2]. KD is unusual in adults. A high proportion of HIV infection is found in adults, especially in recurrent forms. Clinical features are similar in adults and children, but articular involvement and lymphadenopathy occur more frequently in adults, as well as elevated liver enzymes levels and eosinophilia. Coronary artery aneurysms seem to be less frequent than in children [3]. Treatment should be started in the acute phase of the illness, before skin desquamation, in order to obtain significant benefits from the therapy [3]. Steroid therapy is controversial, because of its potential role in coronary aneurysms formation [4]. Renal involvement is rare. Few cases of KD with acute renal failure have been described, and only in children. These cases include three tubulointerstitial nephritis [5], and one mesangial hypercellularity with interstitial infiltrate and immune deposits within glomeruli [6]. Rhabdomyolysis or heart failure are the other aetiologies of acute renal failure in this setting. A case of secondary renal artery stenosis [7] and a case of pelviureteric junction obstruction have also been described [8]. No worsening of renal function with intravenous immunoglobulin treatment has been reported in adult KD. To our knowledge, this is the first report of acute renal failure in an adult KD. The clinical and biological findings were consistent with tubulo-interstitial involvement, which began to improve spontaneously and while treated by intravenous immunoglobulin. This case shows that adult KD should be considered as a cause of acute renal failure with multisystemic features, underscoring the role of aspirin and early intravenous immunoglobulin treatment, and the potential worsening effect of steroid therapy. Conflict of interest statement. None declared.