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Mucocutaneous lymph node syndrome represents a series of clinical findings that has been observed primarily in Japanese children. The disease now appears to be migrating to this country. It involves the cervical lymph nodes, the skin, and mucus membranes. Although the course is usually benign and self-limiting, a number of deaths have resulted from coronary artery disease.
Mucocutaneous lymph node syndrome (MLNS) is a recently described disease entity of unknown etiology that mainly affects children. The disease is characterized by fever, oral mucosal changes, cervical lymphadenopathy and a peripheral skin rash with induration of hands and feet and subsequent desquamation. Thromboarteritis of the coronary vessels causes death in 1 to 2 percent of the patients. Over 7,000 cases have been reported in Japan and increasing numbers are reported in the United States. No specific treatment is available.
Mucocutaneous lymph node syndrome (MCLS) in the young has been known to have coronary aneurysms, and then it has been recently suspected as a cause of premature atherosclerosis and cardiomyopathy. Thirty-three schoolchildren who suffered from MCLS were studied to evaluate cardiac involvements and left ventricular function using two-dimensional (2-D) echocardiograms and submaximal stress test. Fifteen normal schoolchildren were studied as normal control. All these MCLS children were asymptomatic and had no significant findings in routine chest X-ray and electrocardiographic examinations. According to submaximal stress test, 8 cases showed a J type ST depression of only 0.5--1.0 mm, and there were no positive cases. Using 2-D echocardiograms, the left coronary artery was detected in 85% and the right coronary in 27%. One case showed an aneurysm of the left coronary artery. However, none of them showed abnormal left ventricular wall motion or the wall motion abnormality compatible with cardiomyopathy. There were no significant differences between MCLS and normal control in ejection fraction, mean VCF, diastolic descent rate of the anterior mitral valve, D/S ratio of the left ventricular wall, and Weissler's index (PEP/ET). These findings suggested that 1) most of MCLS schoolchildren do not have obvious cardiac involvement and their left ventricular function is within normal limits, 2) because of its low sensitivity, submaximal stress test is not so useful in screening coronary lesions, and 3) the 2-D echocardiogram works not only in detecting coronary aneurysms but also in evaluating left ventricular function.
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 sometines 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 serium 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.
PURPOSE: To report anterior uveitis as the initial sign of adult Kawasaki syndrome (mucocutaneous lymph node syndrome). METHODS: Case report. RESULTS: Kawasaki syndrome was diagnosed in an 18-year-old woman with reduction of vision caused by anterior uveitis, fever, erythemateous cutaneous rash, conjunctival injection, and cervical lymph adenopathy, after medical examination including serologic tests. Aspirin and intravenous immunoglobulin were given, resulting in improvement of the condition. CONCLUSION: Slit-lamp examination should be useful in the evaluation of patients with suspected Kawasaki syndrome, differentiating this condition from streptococcal and staphylococcal toxin-mediated diseases, viral infections, and drug reactions, not commonly associated with anterior uveitis.
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Mucocutaneous lymph-node syndrome (MCLS) is an acute exanthem with specific clinical features, sometimes complicated by involvement of internal organs. Two patients with MCLS had clinical and radiographic evidence of mechanical small-bowel obstruction, probably on the basis of focal vascular insufficiency, as anatomic obstruction was not documented in either instance. The cases indicate that intestinal involvement in patients with MCLS can simulate intestinal obstruction, and conservative management of such patients is appropriate.
Kawasaki disease (mucocutaneous lymph node syndrome) (MCLS) is an apparently infectious disease, an etiological agent of which has not been established, with peak age incidence at about 1 year, but with progressively fewer cases occurring into the fourth decade. Early clinical features include fever, rash, conjunctival injection, dry reddened lips, oropharyngeal reddening, enlarged cervical nodes, and swelling and redness of hands and feet. Peeling of skin of fingers and toes, arthralgia, and marked thrombocytosis are frequent 1-2 weeks after onset. Myocarditis, cardiac valvulitis, and lymphocytic or mixed interstitial infiltration of pancreas, renal, splenic, and hepatic hilar regions are seen in the early phase, but arteritis, typically of extraparenchymal arteries, is the most important aspect of MCLS, hence the term infantile periarteritis nodosa, formerly applied to fatal cases of MCLS. Thrombosis of coronary artery aneurysms is the most common cause of death (rate about 0.5%). The peak time of death is 3-4 weeks from onset, but death from coronary occlusion has been seen as late as 14 years after the acute phase. Aneurysmal rupture with hemopericardium or retroperitoneal hemorrhage is rare, as are late brachial, iliac, or other arterial aneurysms. Pathological features of MCLS in the early and later stages are described and illustrated, and the epidemiologic, etiologic, forensic, and other aspects of the disease are discussed.
Mucocutaneous lymph node syndrome has rarely been reported in the dental literature despite the orofacial features characteristic of the disease. A case is reported in which the cardinal signs were present: erythema of the oral mucosa, cervical lymphadenopathy, conjunctivitis, pyrexia, and desquamation of the skin of the hands and feet. In addition, hydrops of the gallbladder and cardiac disorder were also found. The patient may first seek treatment by the dental practitioner, by whom the diagnosis may be made. Attention is drawn to the possibility of cardiac abnormalities that may influence dental treatment.
Mucocutaneous lymph node syndrome (MLNS) is a newly recognized entity, widely prevalent in Japan. It has also been reported in Korea, Hawaii, and Greece. We have recently seen four white children, 3 to 4 1/2 years of age, with MLNS, in Tucson, Ariz. They had all the principal signs and symptoms characteristic of MLNS.
The diagnosis of mucocutaneous lymph node syndrome is based on the clinical picture as much as on the laboratory findings. It is often considered late in the course of illness. Enteroviral infections and mucocutaneous lymph node syndrome have several common clinical features. Inability to recognize this syndrome can result in misdiagnosis of a viral syndrome with aseptic meningitis.
Clinical findings associate mucocutaneous lymph node syndrome (MCLS) with diffuse arteritis leading to the formation of large aneurysms. Ultrasound assists in the diagnosis of the associated aneurysms, and aids the clinical management of the patients.
Kawasaki syndrome is a newly-recognized clinical entity characterized by multisystem involvement. It has an acute onset and triphasic clinical course. Although essentially a self-limiting disease, permanent vascular damage, especially involving the coronary arteries, may result. Pathologically, the disease is characterized by widespread vasculitis. There is a monomodal age distribution, with peak occurrence during the first two years of life and few affected over the age of 8 years. Males outnumber females 1.5:1; persons of Japanese extraction are overrepresented compared with other races, caucasians underrepresented. Community-wide epidemics have been reported from diverse locations but there is no evidence for direct person-to-person transmission. Etiology remains unknown. Therapy is supportive and should be directed at careful clinical evaluation for cardiovascular abnormalities and antiplatelet aggregation therapy.
Kawasaki Syndrome is a newly recognized clinical entity characterized by multisystem involvement. It has an acute onset and a triphasic clinical course. Although essentially a self-limited disease, permanent vascular damage, especially involving the coronary arteries, may result. Pathologically the disease is characterized by widespread vasculitis. There is a monomodal age distribution with peak occurrence during the first 2 years of life; few affected over the age of 8 years. Males outnumber females 1.5:1, persons of Japanese extraction are overrepresented compared with other races, and Caucasians are underrepresented. Community-wide epidemics occur in diverse locations but there is no evidence for direct person-to-person transmission. Etiology remains unknown. Therapy remains supportive and should be directed at careful clinical evaluation for cardiovascular abnormalities and antiplatelet aggregation therapy.
Mucocutaneous lymph node syndrome has been reported to occur only in children. Three adults aged 16 to 27 years had findings compatible with this illness. Patients with fever, polymorphous skin eruption, congested conjunctiva, reddened palms and soles, red lips and oral mucous membrane, and soft-tissue swelling of the peripheral extremities and who experience membranous desquamation of fingers and toes should be suspected of having mucocutaneous lymph node syndrome. No laboratory test is currently available for confirmation of the diagnosis.
Since infantile mucocutaneous lymph node syndrome was first reported in the United States in 1974, a number of cases of so-called Kawasaki syndrome have been reported in adults. A patient with characteristics of both mucocutaneous lymph node syndrome and toxic shock syndrome is described, and 12 cases reported in the American literature are reviewed in an attempt to clarify the differential diagnosis. Most cases initially reported as Kawasaki syndrome are probably toxic shock syndrome.