Statistics from Altmetric.com
We report a case of Kawasaki disease (KD) following meningococcal septicaemia which we believe has not been described before. A 14 month old boy presented to his local hospital with a four day history of being unwell, fever, and blanching maculopapular rash. Meningococcal septicaemia was diagnosed clinically and the boy was managed with fluid support and intravenous antibiotics. His recovery was complicated by developing respiratory syncytial virus positive bronchiolitis and secondary surgical emphysema. Polymerase chain reaction) was positive for group B meningococcus on day 3. Blood and urine cultures were negative. He continued to spike high temperatures in the ward; a lumbar puncture performed on day 13 showed normal cerebrospinal fluid microscopy and biochemistry. Other investigations, including cranial computed tomography scan of his brain and abdominal ultrasound (including renal vessel Doppler studies) were all normal. He continued to spike high temperatures with pleomorphic erythematous rash, non-purulent conjunctivitis, red enlarged lips, red gums, red inflamed tongue, and axillary lymphadenopathy >1.5 cm. A clinical diagnosis of KD was made; he was treated with intravenous immunoglobulin and aspirin with good effect. Platelet count on day 14 was 933 (admission platelet count was 187). On day 18 he was noted to have mild peeling of his scrotum, hands, and feet. An echocardiogram showed left coronary artery ectasia. He was discharged on day 22 with follow up arrangements including repeat echocardiogram. He was, however, lost to follow up and no further data are available.
A number of epidemiological and clinical observations suggest that KD may be caused by an infectious agent. These include geographic clustering of outbreaks, often with a seasonal predominance and the acute self limited nature of the illness. Many of the clinical features of KD are similar to those of other infectious diseases, for example, adenoviral infection and scarlet fever. Staphylococci, streptococci, and Epstein–Barr virus are some of the infectious agents implicated in KD.1 An unusual degree of immune activation caused by bacterial and viral protein toxins acting as superantigens is currently considered to be the basis of pathology in KD.2–4 We believe that our case shows the possibility that a meningococcal toxin could act as a superantigen to cause KD. We were unable to find any published record of such an association in the literature. The currently proposed hypothesis to explain the pathogenesis is that a genetically susceptible host becomes colonised on the mucous membranes of the gastrointestinal tract by an organism that produces a toxin which behaves as a superantigen. We propose that a toxin producing meningococcus could cause KD in the same fashion as toxic shock syndrome toxin producing Staphylococcus aureus. It is possible that our patient coincidentally had both illnesses at around the same time. Understanding the aetiology of KD remains a major unresolved issue in paediatrics. Although there is no conclusive data to support the superantigen induced disease theory for KD, evidence suggesting that superantigens may mediate KD is growing.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.