P7

LYMPHADENITIS AS DOMINANT PRESENTING MANIFESTA- TION OF KAWASAKI DISEASE. Julle J. Klm and Stanford T. Shulman. Children's Memorlal Hospi- tal, Northwestern U. Med. Sch., Chicago, If.

Cervical adenopathy is the least common cri- terion of Kawasakl Disease (KD), present in - 50t of cases. We report 8 KD patients for whom medl- calattentlon was sought primarily or solely because of striking cervical lymphadenopathy. Pa- tlents ranged from 2-9 yrs. old (median = 5 yrs); 6 were boys and 2 were girls. Initial diagnoses were suppurative lymphadenltls (n=7) and/or tor- ticollls (n=3). Fever and leukocytosls were con- sistent findings. CT imaging was performed in 4 pts.; 3/4 scans showed increased retropharyngeal space and perltonslllar or parapharyngeal lymph- adenopathy with areas of low density suggesting abscess formation. Three pts. underwent lymph node aspiration and one had aspiration of the retropharyngeal space. In each case, aspirates were negative. Parenteral antibiotics were given to 7 patients without improvement. The diagnosis of KD was delayed in most cases, being made 1-13 days (median - i0 d.) after onset of fever. IVIG and ASA were given to 6 patients with rapid reso- lutlon of signs and symptoms, including lymphade- nopathy. Patients with KD may initially present with findings suggestlng bacterial lymphadenltls with or without other manifestations of KD. They should be followed closely for development of other signs of KD. Earlier diagnosis of KD should alleviate the need for unnecessary procedures and antibiotics and lead to more timely therapy di- rected at KD.

P8

TWO CASES OF KAWASAKI DISEASE WITH SEVERE PERIPHERAL ISCHEMIA

Hiroko Kurozumi, Masaaki Mori, Kunihiko Akagi

Severe peripheral ischemic change, including necrosis of the finger tips and toes, occurs infrequently in patients w i th Kawasaki disease. We observed this complication in 2 patients, and carried out a postmortem examination in one of them. <Case 1 > The patient was a 1-month-old male. He developed fever, rash, and generalized edema. Kawasaki disease was suspected and treatment was started on the second day of illness. Dilatation of the corona~ arteries and DIC was noted on.the 6th day of illness. Whde high fever, edema, and hepatosplenomegaly persisted, desquamation of the finger tips occurred on the 7th day. On the 14th day, Raynaud's phenomenon appeared in the f inger tips, and coronary aneurysms, hype r t ens ion , p r o t e i n u r i a , and hypocomplementemm were noted. Necrosis of the finger tips subsequently occurred and he died of heart failure on the 33rd day. The postmortem examination revealed giant aneurysma and thrombi in the coronary arteries, ascending aorta, bilateral axillary arteries, and iliac arteries as well as focal infarction of the liver and kidneys. Histological examination revealed complex lesions involving arteritis of both the large and small arteries, and arteritis as well as partial fibrosis and granulation were also found. <Case 2> The patient was a 7-month-old female. Kawasaki disease was diagnosed due to the presence of typical symptoms. Desquamation occurred on the 7th day, while high fever was persisted. Hepatosplenomegaly, oliguria, and DIC developed on the 13th day, followed by coronary aneurysms, hematuria, and hypocomplementemia despite the various treatment regimens tried. Raynaud's phenomenon was seen in the finger tips on the 20th day. Although necrosis of the finger tips was avoided by using PGEI, she died of a ruptured abdominal aortic aneurysm on the 34th day of illness.

We also investigated the association of Kawasaki disease with IPN and infantile KussmauI-Maier PN.

PSORIATIC RASH IN KAWASAKI DISEASE Robert P. Sundel, Jane C. Burns, Jane W. Newburger, and Stephen E. Gellis, Children's Hospital, Boston, MA.

P9

The skin rash of Kawasaki Disease (KD) is clinically polymorphous, and histologically notable for extensive edema of the papillary dermis. Vesicular or bullous lesions are considered to be extremely unusual. We now report six children with KD who developed an eruption during their illness with features of psoriasis that persisted late Into the convalescent phase. The six children were seen by a single dermatologist (SEG) between 12/86 and 6/90. At the onset of KD, they ranged In age from 7 to 57 months; one of the six had a family history of psoriasis. All were treated with intravenous gamma globulin (IVGG), 1.6-2.0 g/kg. In each case, the rash began as a diffuse erythematous maculopapular eruption, but within several days evolved to Include edematous plaques studded with micropustules. Psoriatic plaques that often involved the face were prominent during the convalescent phase. Biopsies were obtained in two cases, and these were consistent with psoriasis. The course of the KD was unusually severe in the children who developed a psoriasiform eruption. Fever persisted or recurred after treatment with IVGG in four of the six, and three of these developed coronary artery abnormalities. One additional child was noted to have severe sensorineural hearing loss following the acute episode of KD. To date, none of the children has developed chronic psoriasis. We conclude that a psoriasiform skin rash may be the sole or prominent cutaneous manifestation of KD; this type of rash may be associated with a more severe disease course.

PIO

RECOGNITION OF ATYPICAL KAWASAKI DISEASE

Amin Kabani, Ari Joffe, Mireille LeMay, Taj Jadavji, Alberta Children's Hospital, University of Calgary, Calgary, AB, CANADA

Reports of atypical Kawasaki Disease (KD) in the last decade have increased and there is a suggestion that their complication and mortality rate is higher than in typical disease. However, therapy is often delayed due to late diagnosis. In a retrospective review of KD at our hospital from March 1980 to April 1991 we identified 60 consecutive cases of KD, 10 with atypical KD. In atypical KD the M:F was 2.3:1, with mean age 16.6 mos, median age 10.5 mos; in typical KD the M:F was 1.5:1, with mean age 40 mos, median age 31.5 mos. Atypical KD occurs in a significantly younger age group, and has a markedly lower incidence of cervical adenopathy and peripheral changes, with more "patchy" mucositis. Atypical KD has all the laboratory changes of KD, with a significantly higher coronary artery comlication (CAC) rate.

Atypical Typical Atypical Typical fn=10) (n=50) fn=10) fn=501

Mucositis<l site 60% 10% WBC 24A 17.7 No mucositis 20% 0% Hg 90.3 102.9 Rash 100% 100% ESR 78 89.9 Nodes 0% 72% Platelets 889 758 Conjunctivitis 60% 98% tX/uHa 80% 62% Peripheral 30% 84% CAD llYTo 2% Diarrhea 60% 46% CAA 50% 20% Elevated LFTs 40% 44% Irritable 80% 80%

Recognition of atypical KD remains a problem, but knowledge of " the clinical features and laboratory changes should help in early recognition and therapy to prevent the high complication rate.