All issues > Volume 19(4); 1976

Case Report

J Korean Pediatr Soc. 1976;19(4):295-300. Published online April 30, 1976.

Infantile acute febrile Mucocutaneous Lymph Node Syndrome

Hyung Ki Ko¹, Tai Hyuk Yim¹, Don Hee Ahn¹, Keun Chan Sohn¹

¹Department of Pediatrics,National Medical Center, Seoul, Korea

Abstract

Infantile acute febrile Mucocutaneous lymph node syndrome was first described as a new clinical entity by Kawasaki in Japan in 1967. It is characterized by fever, conjunctival congestion, reddening of lips and oral cavity, strawberry like tongue and accompanied by non suppurative swelling of cervical lymph nodes. The author insisted that this entity was quite distinct from erythema multiforme, infantile periarteritis nodosa, tevensJohnson syndrome and scarlet fever in many ways. Since then, nearly 7, 000 cases were reported in Japan until 1975. In Korea 8 cases were reported in 1973. There have been also 12 case reports from Hawaii, 3 from continental United States and 8 from Greece. We experienced 2 castes of such patients in 3 year old boy and 11 months old female infant respectively. Their clinical symptoms, laboratory data and clinical courses were presented com- paring to originanal article with brief review of other literatures. Case I. A 3 year old, Korean male admitted due to high fever and skin rash. One day PT A, he suffered from abdominal pain. In the morning of admission day, skin rash began to appear on the distal part of extremities. Past and family history were not contributory. Vital signs showed BT 39.2C and pulse 168/min. On physical exam, erythematous polymorphous maculopapular skin rash on the whole body, congested conjunctiva, dry and reddened lips, protruded tongue palillae and right cervical lymphnode enlargement were noted. Mild jaundice and diarrhea were also noted. Lab. findings showed Hb 11. igm%. ESE 28，WBC 15300(Band neut 41%, Seg neut 48%, Lympho 5%, Mono 3%, Metamyelocyte 3%) and mild proteinuria. Throat swab culture, blood, urine and stool culture were negative. Chest X-ray was negative. On the 7 th day of illness, skin rash was almostly subsided and membranous desquamation noted on 13 th day of illness. Case I. An eleven months old, Korean female admitted due to fever and rash. 4 days PTA, he was noted high fever, followed by swelling of bilateral cervical region. 2 days PTA, skin rashes were noted at ost auricular area and lips became dry and reddened. Past historj showed that she was prone to catch cold. Vital signs were BT 39.7°C and pulse 124/min. On physical exam, erythematous polymorphous maculopapular skin rashes were noted on the whole body. Lips were dry, reddish and fissuring. Walnut sized bilateral cervical lymphnodes were palpable. Palms and soles were reddened with indurative edema. Laboratory findings showed, Hb 13. 3 WBC 31500 (Seg. neut 51%, lympho 28%, banded neut 12%, mono 6%) and ESR 45. Throat swab culture and urinalysis were negative. AST was 20. Electrophoresis showed increased alpha-2 globulins. On the 1st week of illness, fever and indurative edema of hand were almostly subsided. On the 10 th day of illness, skin rashes were also almostly disappeared. On the 12 th day of illness, desquamation of skin was noted.

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