Kawasaki Disease Mucocutaneous Lymph Node Syndrome

Kawasaki disease, or mucocutaneous lymph node syndrome (MLNS) is a disease of unclear etiology found predominantly in children under 9 years of age.

The diagnosis of this disorder is based on a constellation of clinical findings. The patient must exhibit a prolonged fever associated with at least four of the following: (1) conjunctivitis, (2) rash, (3) lymphadenopathy, (4) changes in the oropharynx consisting of injection of the pharynx and lips with prominent papillae of the tongue (strawberry tongue), and (5) extremity erythema and edema.

The rash has been described as erythematous, morbilliform, urticarial, scarlatiniform, or erythema multiforme-like. It has a predilection for the perineum. Additional supportive evidence which may help in the presumptive diagnosis are leukocytosis, elevation of acute-phase reactants, elevated liver function tests, arthritis, arthralgia, and irritability.

In the second phase, there is usually a sharp rise in the platelet count, desquamation of the fingers and/or toes, and the most serious complication, the development of coronary artery aneurysm. A small percentage (1 to 2 percent) of patients with coronary artery aneurysm develop sudden cardiac failure, resulting in death from myocardial infarction with coronary artery thrombosis.

The differential diagnosis includes drug allergy, toxic epidermal necrolysis, staphylococcal toxin-mediated syndromes, erythema multiforme, and scarlet fever. The etiologic speculations include a hyperimmune response to a variety of infections, a viral syndrome, allergic or toxic response to pollutants, drugs, toxic agents, and a possibility of a rickettsial disease.

Treatment of Kawasaki disease is controversial and includes various antibiotics, salicylates, and steroids. Intravenous gamma globulin is now routinely recommended. Aspirin may be the most promising therapy. Bed rest, supportive therapy, and frequent monitoring are mainstays of treatment.

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