Abstract
Extract: Mucocutaneous lymph node syndrome (MCLS) is a newly recognized disease characterized by fever persisting for more than 5 days, an erythematous skin eruption, conjunctival congestion, dry red fissured lips, reddened tongue, palms, and soles, nonpurulent lymphadenopathy, and sometimes diarrhea, arthralgia, and aseptic meningitis. Additional features may include carditis, pericarditis, aneurysmal dilation and thrombosis of coronary arteries, and sudden death. There is a striking similarity of fatal cases to infantile polyarteritis nodosa, a disease recently reported to be associated with elevated levels of serum IgE. Indeed, it is likely that MCLS represents a disease which can progress to polyarteritis nodosa in infants and young children.
The paired acute and convalescent serum IgE levels of 20 subjects with acute nonfatal MCLS were studied along with 20 near-age unaffected controls from the same communities in Japan. The results indicate that most if not all subjects with MCLS in the study had an elevation of total serum IgE during the acute phase of the disease (geometric mean 157 IU/ml compared with the control value of 38 IU/ml, P = 0.005). The level appeared to reach a peak 1–2 weeks after onset and declined over the ensuing 1–2 months.
Speculation: It may be that only subjects who are genetically programmed for a brisk IgE response to either a rickettsial-like agent or to some other unidentified immunogen will develop the usual manifestations of the disease. Those with persistently elevated IgE levels may be the most likely to develop polyarteritis and hence the most susceptible to serious cardiovascular complications. Specific IgE antibodies may be important in the pathogenesis of the disease. These could bring about the release of chemical mediators which in turn could increase vascular permeability and facilitate antigen-antibody deposition and leukocyte migration in areas such as the arteriolar intima where the pathology chiefly occurs and where rickettsial-like bodies have reportedly been found.
Since serum IgE levels may reflect disease activity in MCLS prospective studies should be carried out in conjunction with sedimentation rates, electrocardiograms, roentgenographic studies, etc., to determine the clinical utility of serial serum IgE levels in following children during and after the acute phase of the disease.
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Kusakawa, S., Heiner, D. Elevated Levels of Immunoglobulin E in the Acute Febrile Mucocutaneous Lymph Node Syndrome. Pediatr Res 10, 108–111 (1976). https://doi.org/10.1203/00006450-197602000-00007
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DOI: https://doi.org/10.1203/00006450-197602000-00007
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