Department of Medical Pathophysiology, University La Sapienza, Rome and 1Department of Infectious and Tropical Diseases, Policlinico S. Matteo, University of Pavia, Pavia, Italy
Correspondence to:
G. Barbaro, Viale Anicio Gallo 63, 00174 Rome, Italy. E-mail: g.barbaro{at}tin.it
SIR, Kawasaki disease is an idiopathic vasculitis typically seen in children less than 5 yr of age [1]. Adult HIV-infected patients present with a constellation of signs and symptoms very similar to those of non-HIV children, except that gastrointestinal complaints are more common and cervical lymphadenopathy less pronounced [2]. Investigations of rare clinical syndromes are difficult, as they require either the incidental presentation of an affected individual to clinicians with an intimate knowledge of the disease or well-organized multicentre collaborations that can capture rare events. Because the first of these requirements was fulfilled, we were able to to investigate a Kawasaki-like syndrome in an HIV-infected adult.
A 34-yr-old HIV-infected homosexual male presented to the hospital with an illness of 1 week that began with fevers, sore throat, fatigue, malaise, anorexia, diffuse abdominal pain, nausea and arthralgias. He subsequently developed conjuctival injection, a diffuse pruritic skin rash involving the trunk and extremities, and painful erythema/swelling of his hands and feet. His past medical history was remarkable only for HIV infection, diagnosed in 1996. He had been on stavudinedidanosine (DDI) indinavir since 1999. His most recent CD4 count was 512 cells/mm3 and the viral load was 850 copies/ml. Results of his physical examination included conjuctival injection without exudate, injection of the oral pharynx without exudate, tender cervical adenopathy (<1 cm), painful erythema/swelling of his hands and feet, and a diffuse macular rash involving the trunk and extremities. He was tachycardic without pathological murmur, his spleen was mildly enlarged, and his joints were unremarkable. He had daily fevers in hospital, with maximum temperature of 38.4°C.
On admission, his antiretroviral therapy was stopped, laboratory studies were performed and a skin biopsy was taken before therapeutic intervention. Because the patient was a Jehovah's Witness and refused all blood products, he was started on a course of methylprednisolone [40 mg intramuscularly twice daily] and aspirin (10 mg/kg once daily) for 1 week rather than standard Kawasaki disease therapy with intravenous immunoglobulin (Ig) and aspirin. He defervesced on the third day of methylprednisolone therapy. The patient continued methyprednisolone at 20 mg daily intramuscularly for an additional week and aspirin at a reduced dose (160 mg daily). After 1 week of therapy, all the patient's protean symptoms had resolved, including the sore throat, pain in his hands and feet and pruritic rash. Periungal peeling was noted on a follow-up out-patient clinic visit 17 days after admission to hospital. At 6 weeks he was symptom-free except for some residual fatigue. Antiretroviral therapy was restarted with a regimen of stavudinelamivudine efavirenz, and low-dose aspirin therapy (160 mg daily) was continued.
Table 1 summarizes the immunological data collected before initiation of steroid therapy and 6 weeks after hospital discharge. Our patient had a selective elevation of serum IgA on presentation that had not returned to a normal value 6 weeks later. Cytokine analysis revealed acute elevations of interferon (IFN)- and IFN-
that resolved within 6 weeks. An extensive evaluation for potential infectious aetiologies (including EpsteinBarr virus, cytomegalovirus, parvovirus-19 and human herpesvirus-6) was negative. Skin biopsy showed an infiltrate of lymphocytes, neutrophils and IgA plasma cells surrounding the superficial capillary plexus in the papillary dermis, with destruction of small venules and moderate fibrinoid necrosis. Immunohistochemistry of a skin biopsy sample is shown in Fig. 1.
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From a therapeutic perspective, a distinction between the syndromes is not critical as the treatments for pediatric Kawasaki disease and adult HIVKawasaki-like syndrome are identical. HIV-infected patients with Kawasaki-like syndromes respond to intravenous Ig and aspirin, the standard therapy for paediatric Kawasaki disease [2]. As in the paediatric syndrome, steroids appear to be a reasonable alternative for HIV-infected patients unable to be treated with standard immunoglobulins. Though it is somewhat counterintuitive, short-term steroids are not likely to be detrimental to the control of HIV viraemia. Immunohistochemical evaluation for IgA plasma cells in skin biopsies may be a useful adjunct in making a diagnosis of a Kawasaki-like syndrome in an HIV-infected adult. Although our data suggest a common pathophysiology, final resolution of the relationship between these syndromes awaits identification of the aetiological agent of paediatric Kawasaki disease.
The authors have declared no conflicts of interest.
References