mucocutaneous lymph node syndrome (MLNS), an acute febrile illness, primarily of young children, characterized by inflamed mucous membranes of the mouth; “strawberry tongue”; cervical lymphadenopathy; polymorphous rash on the trunk; and edema, erythema, and desquamation of the skin on the extremities. Other commonly associated findings include arthralgia, diarrhea, otitis, pneumonia, photophobia, meningitis, and electrocardiographic changes. The cause is unknown. No clear-cut environmental, seasonal, or geographic factors have been discovered, and person-to-person transmission is unproved. A genetic predisposition has been indicated. Treatment includes IV gammaglobulin; aspirin in large doses, which may be prescribed over a long period; and supportive care. Also called Kawasaki disease. ▪ OBSERVATIONS: The acute stage of the disease begins with a remittent fever (103° F to 105° F) accompanied by extreme irritability, lethargy, and intermittent colicky abdominal pain. The fever typically lasts 7 days to 2 weeks and is unresponsive to antibiotics. After 1 to 2 days, bilateral conjunctivitis occurs. Within 5 days, there is erythema and edema of the hands and feet, a macular rash on the trunk and perineum, strawberry tongue, fissuring of the lips, and reddened pharynx. Cervical lymphadenopathy is present. Ten to 25 days after onset, fever resolves and symptoms diminish. Irritability, anorexia, and conjunctivitis remain. There is peeling of the hands and feet, arthritis, arthralgia, and thrombocytosis. The greatest risk for coronary artery aneurysm occurs during this time. During convalescence, all clinical signs have resolved, but erythrocyte sedimentation rate is still abnormal. ESR blood values return to normal about 6 to 8 weeks after onset. Clinical exam reveals a history of fever with four of five of the following conditions: conjunctivitis, changes in mucosa of oropharynx, edema and erythema of hands and feet, nonvesicular truncal rash, and lymphadenopathy. Blood studies reveal elevated WBCs and platelets; elevated ESR; positive C-reactive protein; and elevated ALT and AST. Chest x-rays may reveal pulmonary infiltrates. An echocardiogram may show depressed left ventricular function. Echocardiograms are also useful to monitor cardiac status during the course of the disease. Complications include coronary arteritis, coronary artery aneurysms, and thrombotic occlusion of an aneurysm leading to a myocardial infarction and possible death. However, prognosis with treatment is excellent, with a mortality rate of less than 0.1%. ▪ INTERVENTIONS: Intravenous immunoglobulin is the primary treatment to reduce inflammation in the acute stage. Aspirin therapy is used until temperatures return to normal. Corticosteroids increase possibility of aneurysm and are contraindicated in treatment of MLNS. Anticoagulant and antiplatelet therapy may be used if there is evidence of coronary thrombosis. If the child develops cardiac complications, percutaneous transluminal coronary angioplasty, coronary bypass grafting, or cardiac transplant may be required. ▪ PATIENT CARE CONSIDERATIONS: Nursing care is primarily supportive and includes careful monitoring of cardiac status, careful fluid replacement, I & O, comfort measures (lubrication for lips, lotion for skin, and cool cloths), quiet soothing environment, and parental support in attempting to console an irritable child. Parental education is needed about possible cardiac complications. Caregiver instruction in CPR should be provided if cardiac complications are present.
