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Acute Febrile Mucocutaneous Lymph Node Syndrome FREE

Richard Yanagihara, MD; James K. Todd, MD
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Am J Dis Child. 1980;134(6):603-614. doi:10.1001/archpedi.1980.02130180059017.
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More than a decade ago, Tomisaku Kawasaki, a Japanese pediatrician, described an acute exanthematous disease characterized by persistent fever, mucous membrane hyperemia, cervical lymph node enlargement, and periungal desquamation in 50 infants and children who had been seen during the preceding six-year period at the Japan Red Cross Medical Center in Tokyo.1 Individually the clinical and laboratory features seemed not unlike those of other mucocutaneous syndromes and exanthematous illnesses of childhood, but Kawasaki decided that the constellation of findings was distinctive and constituted a hitherto undescribed clinical entity. The disease was designated mucocutaneous lymph node syndrome (MCLS).1

After this description, numerous cases were reported throughout Japan, and in 1970 the Research Committee of MCLS, supported by the Ministry of Health and Welfare of the Japanese government, was organized.

The primary purpose of this group was to conduct and to coordinate investigations involving the etiology and the clinical, pathological,

REFERENCES

Kawasaki T:  Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children: Clinical observations of 50 cases (in Japanese) . Jpn J Allergol 16:178-222, 1967;.
Fetterman GH, Hashida Y:  Mucocutaneous lymph node syndrome (MLNS): A disease widespread in Japan which demands our attention . Pediatrics 54:268-270, 1974;.
Hussey HH:  Mucocutaneous lymph node syndrome . JAMA 236:292, 1976;.
Riley HD:  Mucocutaneous lymph node syndrome (Kawasaki disease) . J Infect Dis 134:302-305, 1976;.
 Kawasaki disease . Lancet 1:675-676, 1976;.
 Mucocutaneous lymph node syndrome: A new disease . Br Med J 1:407, 1977;.
Hammond BL:  Mucocutaneous lymph node syndrome . Ohio State Med J 73:612-616, 1977;.
Kahn G:  Mucocutaneous lymph node syndrome (Kawasaki's disease) . Arch Dermatol 114:948-950, 1978;.
Morens DM, Nahmias AJ:  Kawasaki disease: A 'new' pediatric enigma . Hosp Pract 13:109-112, 119-120, 1978;.
Terezhalmy GT:  Mucocutaneous lymph node syndrome: Review of a recently described disease complex . Oral Surg 47:26-30, 1979;.
Kawasaki T, Kusakawa S, Shigematsu I (eds): Progress of Research on Kawasaki Disease (MCLS) . Tokyo, Kindai Shuppan Co, 1976;.
Shiokawa Y (ed): Vascular Lesions of Collagen Diseases and Related Conditions . Tokyo, University of Tokyo Press, 1977;.
Becker AE:  Kawasaki disease . Lancet 1:864, 1976;.
Becker AE:  Mucocutaneous lymph node syndrome or infantile polyarteritis nodosa? Pediatrics 60:774, 1977;.
Landing BH, Larson EJ:  Are infantile periarteritis nodosa with coronary artery involvement and fatal mucocutaneous lymph node syndrome the same?: Comparison of 20 patients from North America with patients from Hawaii and Japan . Pediatrics 59:651-662, 1977;.
Aterman K:  A possible early example of mucocutaneous lymph node syndrome . J Pediatr 92:1027-1028, 1978;.
Kato H, Koike S, Sasaguri Y:  Clinicopathological studies of diseases associated with vasculitis: Kawasaki disease (MCLS) and infantile polyarteritis nodosa (in Japanese) . Nippon Rinsho 36:753-767, 1978;.
Yanagawa H:  Epidemiology of Kawasaki disease (in Japanese) . Jpn J Clin Med 34:275-283, 1976;.
Morens DM, O'Brien RJ:  Kawasaki disease in the United States . J Infect Dis 137:91-93, 1978;.
Brown JS, Billmeier GJ Jr, Cox F, et al:  Mucocutaneous lymph node syndrome in the continental United States . J Pediatr 88:81-83, 1976;.
Melish ME, Hicks RM, Larson EJ:  Mucocutaneous lymph node syndrome in the United States . Am J Dis Child 130:599-607, 1976;.
Bitter JJ, Friedman SA, Paltzik RL, et al:  Kawasaki's disease appearing as erythema multiforme . Arch Dermatol 115:71-72, 1979;.
Kawasaki T, Kosaki F, Okawa S, et al:  A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan . Pediatrics 54:271-276, 1974;.
Lauer BA, Bruhn FW, Todd JK, et al:  Mucocutaneous lymph node syndrome in Denver . Am J Dis Child 130:610-612, 1976;.
Robinson R, Adler R:  Mucocutaneous lymph node syndrome in California . J Pediatr 88:1069-1070, 1976;.
Taneja A, Saxena U:  Mucocutaneous lymph node syndrome: Case report . Indian Pediatr 14:927-931, 1977;.
Majeed HA, Olson IA:  Kawasaki disease in Kuwait: A report of two cases . Acta Paediatr Scand 67:525-528, 1978;.
Nyerges G, Barna M, Molnar L:  Mucocutaneous lymph node syndrome: Three cases observed in Hungary . Acta Paediatr Acad Sci Hung 19:1-7, 1978;.
Goldsmith RW, Gribetz D, Strauss L:  Mucocutaneous lymph node syndrome (MLNS) in the continental United States . Pediatrics 57:431-435, 1976;.
Magilavy DB, Speert DP, Silver TM, et al:  Mucocutaneous lymph node syndrome: Report of two cases complicated by gallbladder hydrops and diagnosed by ultrasound . Pediatrics 61:699-702, 1978;.
Bernhardt IB:  Mucocutaneous lymph node syndrome with encephalopathy in the continental United States . West J Med 125:230-233, 1976;.
Cook A, L'Heureux P:  Radiographic findings in the mucocutaneous lymph node syndrome . Am J Roentgenol 132:107-109, 1979;.
Tanaka N:  Kawasaki disease (acute febrile infantile mucocutaneous lymph node syndrome) in Japan: Relationship with infantile periarteritis nodosa . Pathol Microbiol 43:204-218, 1975;.
Tanaka N, Sekimoto K, Naoe S:  Kawasaki disease: Relationship with infantile periarteritis nodosa . Arch Pathol Lab Med 100:81-86, 1976;.
Wentworth P, Silver MD:  Fatal mucocutaneous lymph node syndrome in Canada? Can Med Assoc J 115:299-305, 1976;.
Kegel SM, Dorsey TJ, Rowen M, et al:  Cardiac death in mucocutaneous lymph node syndrome . Am J Cardiol 40:282-286, 1977;.
Kim J, Yeo Y, Lee DB:  Mucocutaneous lymph node syndrome: Clinical observations of eight cases (in Korean) . Korea N Med J 16:1157-1160, 1973;.
Melish ME, Hicks RM, Larson EJ:  Mucocutaneous lymph node syndrome (MCLS) in the US, abstracted . Pediatr Res 8:427, 1974;.
Russell AS, Zaragoza AJ, Shea R:  Mucocutaneous lymph node syndrome in Canada . Can Med Assoc J 112:1210-1211, 1975;.
Radford DJ, Sondheimer HM, Williams GJ, et al:  Mucocutaneous lymph node syndrome with coronary artery aneurysm . Am J Dis Child 130:596-598, 1976;.
Fanconi A, Bodmer P, Egloff B:  Akutes mukokutanes lymph knoten syndrom (Kawasaki syndrom) mit letaler periarteriitis nodosa der kranzarterien . Helv Paediatr Acta 33:135-140, 1978;.
Stephenson SR:  Kawasaki disease in Europe . Lancet 1:373-374, 1977;.
Lyen KR, Brook CGD:  Mucocutaneous lymph node syndrome in two siblings . Br Med J 1:1187, 1978;.
Fossard C, Thompson RA:  Mucocutaneous lymph node syndrome (Kawasaki disease): Probable soluble complex disorder . Br Med J 1:883, 1977;.
Hewitt CJ:  Case of Kawasaki disease . Br Med J 1:883-884, 1977;.
Scopes JW, Hulse JA:  Mucocutaneous lymph node syndrome . Br Med J 1:511, 1977;.
Morgan GJ, Lynch MA:  Kawasaki disease in Canterbury . Arch Dis Child 53:916-917, 1978;.
Carter RF, Haynes ME, Morton J:  Rickettsia-like bodies and splenitis in Kawasaki disease . Lancet 2:1254-1255, 1976;.
Della-Porta G, Alberti A:  Kawasaki disease in Europe . Lancet 1:797-798, 1977;.
Valaes T:  Mucocutaneous lymph node syndrome (MLNS) in Athens, Greece . Pediatrics 55:295, 1975;.
Ahlström H, Lundström N-R, Mortensson W, et al:  Infantile periarteritis nodosa or mucocutaneous lymph node syndrome . Acta Paediatr Scand 66:193-198, 1977;.
Hicsönmez G, Kanra G, Kocak H, et al:  Acute febrile mucocutaneous lymph node syndrome . Clin Pediatr 16:480-481, 1977;.
Smith AD:  Infantile polyarteritis and Kawasaki disease . Acta Paediatr Scand 66:381-384, 1977;.
Corbeel L, Delmotte B, Standaert L, et al:  Kawasaki disease in Europe . Lancet 1:797, 1977;.
Piette N, Carton D, Hooft C:  A case of mucocutaneous lymph node syndrome . Acta Paediatr Belg 30:243-246, 1977;.
Keim DE, Keller EW, Hirsch MS:  Mucocutaneous lymph node syndrome and parainfluenza 2 virus infection . Lancet 2:303, 1977;.
Kovacevic M, Gardner HG:  Thrombocytosis associated with the mucocutaneous lymph node syndrome . J Pediatr 89:688, 1976;.
Dennis MK, Ayoub EM, Graham T, et al:  Mucocutaneous lymph node syndrome in Florida . J Fla Med Assoc 64:21-26, 1977;.
Darby CP, Kyong CU:  Mucocutaneous lymph node syndrome . JAMA 236:2295-2297, 1976;.
Gross CW, Chamberlain M:  Mucocutaneous lymph node syndrome: A new clinical entity . Laryngoscope 87:1702-1704, 1977;.
Miriovsky M, Downing JS:  Mucocutaneous lymph node syndrome . Am Fam Physician 17:154-157, 1978;.
John TJ, DeBenedetti CD, Zee ML:  Mucocutaneous lymph node syndrome in Arizona . Am J Dis Child 130:613-614, 1976;.
Bergeson PS, Schoenike SL:  Mucocutaneous lymph node syndrome: A case masquerading as Rocky Mountain spotted fever . JAMA 237:2299-2302, 1977;.
Bergeson PS, Serlin SP, Corman LI:  Mucocutaneous lymph node syndrome with positive Weil-Felix reaction but negative leptospira studies . Lancet 1:720-721, 1978;.
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Tominaga M, Ohshima K, Yanagawa Y, et al:  An epidemic analysis of family cases of mucocutaneous lymph node syndrome (in Japanese) . Pediatr Japan 18:59-63, 1977;.
Kitayama T:  Infectious disease theory of Kawasaki disease with special reference to the theory of virus infection as a triggering factor (in Japanese) . Jpn J Clin Med 34:295-297, 1976;.
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Yamada K, Fukumoto T, Shinkai A, et al:  The platelet functions in acute febrile mucocutaneous lymph node syndrome and a trial of prevention for thrombosis by antiplatelet agent . Nippon Ketsueki Gakkai Zasshi 41:791-802, 1978;.
Kato H, Koike S, Yamamoto M, et al:  Coronary aneurysms in infants and young children with acute febrile mucocutaneous lymph node syndrome . J Pediatr 86:892-898, 1975;.
Hosaki J, Abe S, Yoshimatsu A, et al:  Observations of coronary arterial lesions in acute febrile mucocutaneous lymph node syndrome (MCLS) . Acta Paediatr Jpn 18:8-17, 1976;.
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Yanagisawa M, Kobayashi N, Matsuya S:  Myocardial infarction due to coronary thromboarteritis following acute febrile mucocutaneous lymph node syndrome (MLNS) in an infant . Pediatrics 54:227-281, 1974;.
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Humphry T, Sanders S, Stadius M:  Leptospirosis mimicking MLNS . J Pediatr 91:853, 1977;.
Ohtaki C, Tomiyama T, Suzuki M, et al:  Leptospiral antibody and MLNS . J Pediatr 93:896, 1978;.
Hamashima Y, Kishi K, Tasaka K:  Rickettsia-like bodies in infantile acute febrile mucocutaneous lymph node syndrome , Lancet 2:42, 1973;.
Tasaka K, Hamashima Y:  Studies on rickettsia-like body in Kawasaki disease: Attempts at the isolation and characterization . Acta Pathol Jpn 28:235-245, 1978;.
Takabatake E:  Etiological studies of Kawasaki disease: A toxicological theory (in Japanese) . Jpn J Clin Med 34:302-305, 1976;.
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Matsuda I, Hattori S, Nagata N, et al:  HLA antigens in mucocutaneous lymph node syndrome . Am J Dis Child 131:1417-1418, 1977;.
Kato S, Kimura M, Tsuji K, et al:  HLA antigens in Kawasaki disease . Pediatrics 61:252-255, 1978;.
Yata J, Nakagawa T, Sawa E:  Some immunological studies on Kawasaki disease , in Shiokawa Y (ed): Vascular Lesions of Collagen Diseases and Related Conditions . Tokyo, University of Tokyo Press, 1977;, pp 335-336.
Kusakawa S, Heiner DC:  Elevated levels of immunoglobulin E in the acute febrile mucocutaneous lymph node syndrome . Pediatr Res 10:108-111, 1976;.
Krous HF, Clausen CR, Ray CG:  Elevated immunoglobulin E in infantile polyarteritis nodosa . J Pediatr 84:841-845, 1974;.
Tasaka K, Hamashima Y:  Function of phagocytosis and intracellular killing of peripheral neutrophils in Kawasaki disease . Acta Pathol Jpn 28:247-252, 1978;.
Kusakawa S:  Treatment of Kawasaki disease (in Japanese) . Jpn J Clin Med 34:306-310, 1976;.
Kato H, Koike S, Yokoyama T:  Kawasaki disease: Effect of treatment on coronary artery involvement . Pediatrics 63:175-179, 1979;.
Nishizawa EE, Wynalda DR, Suydam DR, et al:  Flurbiprofen, a new potent inhibitor of platelet aggregation . Thromb Res 3:577-588, 1973;.
Rich AR, Gregory JE:  The experimental demonstration that periarteritis nodosa is a manifestation of hypersensitivity . Bull Hopkins Hosp 72:65-88, 1943;.

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References

Kawasaki T:  Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children: Clinical observations of 50 cases (in Japanese) . Jpn J Allergol 16:178-222, 1967;.
Fetterman GH, Hashida Y:  Mucocutaneous lymph node syndrome (MLNS): A disease widespread in Japan which demands our attention . Pediatrics 54:268-270, 1974;.
Hussey HH:  Mucocutaneous lymph node syndrome . JAMA 236:292, 1976;.
Riley HD:  Mucocutaneous lymph node syndrome (Kawasaki disease) . J Infect Dis 134:302-305, 1976;.
 Kawasaki disease . Lancet 1:675-676, 1976;.
 Mucocutaneous lymph node syndrome: A new disease . Br Med J 1:407, 1977;.
Hammond BL:  Mucocutaneous lymph node syndrome . Ohio State Med J 73:612-616, 1977;.
Kahn G:  Mucocutaneous lymph node syndrome (Kawasaki's disease) . Arch Dermatol 114:948-950, 1978;.
Morens DM, Nahmias AJ:  Kawasaki disease: A 'new' pediatric enigma . Hosp Pract 13:109-112, 119-120, 1978;.
Terezhalmy GT:  Mucocutaneous lymph node syndrome: Review of a recently described disease complex . Oral Surg 47:26-30, 1979;.
Kawasaki T, Kusakawa S, Shigematsu I (eds): Progress of Research on Kawasaki Disease (MCLS) . Tokyo, Kindai Shuppan Co, 1976;.
Shiokawa Y (ed): Vascular Lesions of Collagen Diseases and Related Conditions . Tokyo, University of Tokyo Press, 1977;.
Becker AE:  Kawasaki disease . Lancet 1:864, 1976;.
Becker AE:  Mucocutaneous lymph node syndrome or infantile polyarteritis nodosa? Pediatrics 60:774, 1977;.
Landing BH, Larson EJ:  Are infantile periarteritis nodosa with coronary artery involvement and fatal mucocutaneous lymph node syndrome the same?: Comparison of 20 patients from North America with patients from Hawaii and Japan . Pediatrics 59:651-662, 1977;.
Aterman K:  A possible early example of mucocutaneous lymph node syndrome . J Pediatr 92:1027-1028, 1978;.
Kato H, Koike S, Sasaguri Y:  Clinicopathological studies of diseases associated with vasculitis: Kawasaki disease (MCLS) and infantile polyarteritis nodosa (in Japanese) . Nippon Rinsho 36:753-767, 1978;.
Yanagawa H:  Epidemiology of Kawasaki disease (in Japanese) . Jpn J Clin Med 34:275-283, 1976;.
Morens DM, O'Brien RJ:  Kawasaki disease in the United States . J Infect Dis 137:91-93, 1978;.
Brown JS, Billmeier GJ Jr, Cox F, et al:  Mucocutaneous lymph node syndrome in the continental United States . J Pediatr 88:81-83, 1976;.
Melish ME, Hicks RM, Larson EJ:  Mucocutaneous lymph node syndrome in the United States . Am J Dis Child 130:599-607, 1976;.
Bitter JJ, Friedman SA, Paltzik RL, et al:  Kawasaki's disease appearing as erythema multiforme . Arch Dermatol 115:71-72, 1979;.
Kawasaki T, Kosaki F, Okawa S, et al:  A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan . Pediatrics 54:271-276, 1974;.
Lauer BA, Bruhn FW, Todd JK, et al:  Mucocutaneous lymph node syndrome in Denver . Am J Dis Child 130:610-612, 1976;.
Robinson R, Adler R:  Mucocutaneous lymph node syndrome in California . J Pediatr 88:1069-1070, 1976;.
Taneja A, Saxena U:  Mucocutaneous lymph node syndrome: Case report . Indian Pediatr 14:927-931, 1977;.
Majeed HA, Olson IA:  Kawasaki disease in Kuwait: A report of two cases . Acta Paediatr Scand 67:525-528, 1978;.
Nyerges G, Barna M, Molnar L:  Mucocutaneous lymph node syndrome: Three cases observed in Hungary . Acta Paediatr Acad Sci Hung 19:1-7, 1978;.
Goldsmith RW, Gribetz D, Strauss L:  Mucocutaneous lymph node syndrome (MLNS) in the continental United States . Pediatrics 57:431-435, 1976;.
Magilavy DB, Speert DP, Silver TM, et al:  Mucocutaneous lymph node syndrome: Report of two cases complicated by gallbladder hydrops and diagnosed by ultrasound . Pediatrics 61:699-702, 1978;.
Bernhardt IB:  Mucocutaneous lymph node syndrome with encephalopathy in the continental United States . West J Med 125:230-233, 1976;.
Cook A, L'Heureux P:  Radiographic findings in the mucocutaneous lymph node syndrome . Am J Roentgenol 132:107-109, 1979;.
Tanaka N:  Kawasaki disease (acute febrile infantile mucocutaneous lymph node syndrome) in Japan: Relationship with infantile periarteritis nodosa . Pathol Microbiol 43:204-218, 1975;.
Tanaka N, Sekimoto K, Naoe S:  Kawasaki disease: Relationship with infantile periarteritis nodosa . Arch Pathol Lab Med 100:81-86, 1976;.
Wentworth P, Silver MD:  Fatal mucocutaneous lymph node syndrome in Canada? Can Med Assoc J 115:299-305, 1976;.
Kegel SM, Dorsey TJ, Rowen M, et al:  Cardiac death in mucocutaneous lymph node syndrome . Am J Cardiol 40:282-286, 1977;.
Kim J, Yeo Y, Lee DB:  Mucocutaneous lymph node syndrome: Clinical observations of eight cases (in Korean) . Korea N Med J 16:1157-1160, 1973;.
Melish ME, Hicks RM, Larson EJ:  Mucocutaneous lymph node syndrome (MCLS) in the US, abstracted . Pediatr Res 8:427, 1974;.
Russell AS, Zaragoza AJ, Shea R:  Mucocutaneous lymph node syndrome in Canada . Can Med Assoc J 112:1210-1211, 1975;.
Radford DJ, Sondheimer HM, Williams GJ, et al:  Mucocutaneous lymph node syndrome with coronary artery aneurysm . Am J Dis Child 130:596-598, 1976;.
Fanconi A, Bodmer P, Egloff B:  Akutes mukokutanes lymph knoten syndrom (Kawasaki syndrom) mit letaler periarteriitis nodosa der kranzarterien . Helv Paediatr Acta 33:135-140, 1978;.
Stephenson SR:  Kawasaki disease in Europe . Lancet 1:373-374, 1977;.
Lyen KR, Brook CGD:  Mucocutaneous lymph node syndrome in two siblings . Br Med J 1:1187, 1978;.
Fossard C, Thompson RA:  Mucocutaneous lymph node syndrome (Kawasaki disease): Probable soluble complex disorder . Br Med J 1:883, 1977;.
Hewitt CJ:  Case of Kawasaki disease . Br Med J 1:883-884, 1977;.
Scopes JW, Hulse JA:  Mucocutaneous lymph node syndrome . Br Med J 1:511, 1977;.
Morgan GJ, Lynch MA:  Kawasaki disease in Canterbury . Arch Dis Child 53:916-917, 1978;.
Carter RF, Haynes ME, Morton J:  Rickettsia-like bodies and splenitis in Kawasaki disease . Lancet 2:1254-1255, 1976;.
Della-Porta G, Alberti A:  Kawasaki disease in Europe . Lancet 1:797-798, 1977;.
Valaes T:  Mucocutaneous lymph node syndrome (MLNS) in Athens, Greece . Pediatrics 55:295, 1975;.
Ahlström H, Lundström N-R, Mortensson W, et al:  Infantile periarteritis nodosa or mucocutaneous lymph node syndrome . Acta Paediatr Scand 66:193-198, 1977;.
Hicsönmez G, Kanra G, Kocak H, et al:  Acute febrile mucocutaneous lymph node syndrome . Clin Pediatr 16:480-481, 1977;.
Smith AD:  Infantile polyarteritis and Kawasaki disease . Acta Paediatr Scand 66:381-384, 1977;.
Corbeel L, Delmotte B, Standaert L, et al:  Kawasaki disease in Europe . Lancet 1:797, 1977;.
Piette N, Carton D, Hooft C:  A case of mucocutaneous lymph node syndrome . Acta Paediatr Belg 30:243-246, 1977;.
Keim DE, Keller EW, Hirsch MS:  Mucocutaneous lymph node syndrome and parainfluenza 2 virus infection . Lancet 2:303, 1977;.
Kovacevic M, Gardner HG:  Thrombocytosis associated with the mucocutaneous lymph node syndrome . J Pediatr 89:688, 1976;.
Dennis MK, Ayoub EM, Graham T, et al:  Mucocutaneous lymph node syndrome in Florida . J Fla Med Assoc 64:21-26, 1977;.
Darby CP, Kyong CU:  Mucocutaneous lymph node syndrome . JAMA 236:2295-2297, 1976;.
Gross CW, Chamberlain M:  Mucocutaneous lymph node syndrome: A new clinical entity . Laryngoscope 87:1702-1704, 1977;.
Miriovsky M, Downing JS:  Mucocutaneous lymph node syndrome . Am Fam Physician 17:154-157, 1978;.
John TJ, DeBenedetti CD, Zee ML:  Mucocutaneous lymph node syndrome in Arizona . Am J Dis Child 130:613-614, 1976;.
Bergeson PS, Schoenike SL:  Mucocutaneous lymph node syndrome: A case masquerading as Rocky Mountain spotted fever . JAMA 237:2299-2302, 1977;.
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