Acute Febrile Mucocutaneous LymphNode Syndrome (Kawasaki Disease) in Adults: Case Report and

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1 Case Report Acute Febrile Mucocutaneous LymphNode Syndrome (Kawasaki Disease) in Adults: Case Report and Review of the Literature Junji TOMIYAMA,Yuuichi HASEGAWA,Yasuo KUMAGAI, Yamao Adachi and Kensuke Karasawa* A 25-year-old female meeting all six criteria for Kawasaki disease is reported. A total of 22 reported cases of adult Kawasaki disease, including the present case, are reviewed. In adult Kawasaki disease, arthralgia, gastrointestinal complications and hepatic dysfunction are seen more frequently than in childhood cases. Cardiac complications are rarely seen in adult Kawasakidisease. Twocases have been positive for anti-nuclear antibody (ANA). The present patient had increased levels of serum IgE and was positive for ANA,suggesting involvement of an immunemechanism. Adult Kawasaki disease is rare but appears to be on the increase; internists treating adults must be aware of this disease. Key words: Adult Kawasaki disease, Anti-nuclear antibody, Immunoglobulin E Kawasaki disease was first reported by Dr. Tomisaku Kawasaki in 1967 and was characterized by persistent fever, mucousmembranehyperemia, cervical lymph node enlargement, exanthema and periungal desquamation (1). Kawasaki disease is almost exclusively an illness of young children, about 80% of the patients are under the age of 4 yr (2). Recently, however, Kawasaki disease has been occasionally reported in adults (3-20). Here, a 25-year-old womanwho fulfilled the clinical criteria for the diagnosis of Kawasaki disease is described. In addition, the 21 cases of adult Kawasaki disease reported are reviewed and the clinical features, etiology and therapy of adult Kawasaki disease are discussed. CASE REPORT A 25-year-old nurse, who was previously in good health, experienced a temperature of 39 C, sore throat, nausea, vomiting and arthralgia. She went to her private physician who prescribed cefteram pivoxil, 600 mg/day, to be taken orally, but her fever persisted, and 2 days later an erythematous, maculopapular, non-pruritic rash was noted on the trunk, arms and legs. She came to Tokyo Metropolitan Bokuto Hospital where she was treated with fosfomycin, 1,000 mg/day, to be taken orally, nevertheless, her fever persisted. Three days later, she developed shotty, non-tender cervical lymphadenopathy and injected conjunctivae. Four days after that, she experienced cough and sputum production, and was admitted to our hospital on July 14, Her menstrual period started on the day of onset of her symptoms, however, she had not used tampons. On admission, her temperature was 38.8 C, pulse 108 and blood pressure 118/70 mmhg.her conjunctivae were injected, the posterior cervical lymph nodes were swollen, her tonsils and posterior pharynx were erythematous, and "strawberry From Departments of Internal Medicine and *Pediatrics, Tokyo Metropolitan Bokuto Hospital, Tokyo Received for publication April 13, 1990; Accepted for publication November 22, 1990 Reprint requests should be addressed to Junji Tomiyama, MD,Department of Internal Medicine, Tokyo Metropolitan Bokuto Hospital, Kotobashi, Sumida-ku, Tokyo 130, Japan 285

2 Tomiyama et al Table 1. Laboratory tongue" was also noted. There was an erythematous maculopapular rash on her legs and marked erythema over her palms and soles. The patient's chest was clear, and a grade II/VI systolic ejection murmurwas heard at the apex. There was no enlargement of the liver or spleen. The neurologic examination was normal. Laboratory data on admission (Table 1) revealed leukocytosis with neutrophilia. Liver function tests were normal, and a2-globulin, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated. Although, antinuclear antibodies (ANA) were slightly elevated, other autoantibodies were negative. Bacterial cultures of blood, throat and urine were negative, but sputum culture yielded Staphylococcus aureus. The chest X-ray showed no abnormalities in the lung fields or cadiac silhouette. ECGrevealed sinus tachycardia but no other abnormalities. Ultrasonography of the abdomenrevealed no hepatosplenomegaly. The patient was treated with aspirin, 2,640 mg, orally, four times daily, and became afebrile on the seventh hospital day. Desquamation of the fingers occurred on the patient's fourth hospital day (Fig. 1). Kawasaki disease was suspected and an echocardiography performed on the eleventh hospital day, but revealed no abnormalities with M-modeor two-dimensional studies and there was no evidence of coronary aneurysm. On the fourteenth hospital day, the aspirin dose was reduced to 660 mg daily and the patient left the hospital 19 days after admission. An echocardiogram examined 1 month later revealed no abnormalities, and aspirin was discontinued. Fig. 1. Periungual desquamation of the skin of the fingers observed eight days after the onset of illness. data on admission 286

3 Kawasaki Disease in Adults DISCUSSION The diagnostic criteria for Kawasaki disease are as follows (1): 1) fever of five or more days duration, 2) changes in the peripheral extremities, such as peripheral edema, peripheral erythema, desquamation, 3) polymorphous exanthem, 4) injected bilateral conjunctiva, 5) changes in the mucous membranes of the oral cavity, such as injected pharynx, injected lips, strawberry tongue, and 6) acute non-suppurative swelling of the cervical lymph nodes. The present patient was diagnosed as having Kawasaki disease based on the presence of all six criteria. The differential diagnosis includes Stevens- Johnson syndrome due to antibiotics, however this can be eliminated on the basis of the absence of changes in the peripheral extremities and the absence of strawberry tongue in the Stevens-Johnson syndrome (21). A diagnosis of toxic shock syndrome (18) must also be excluded. This is unlikely, however, since the patient had no hypotension and Table 2. Reported cases of adult Kawasaki disease failed to respond to antibiotics and because of the fact that there is no lymphadenopathy in toxic shock syndrome. Scarlet fever caused by Staphylococcus aureus (22) should also be ruled out. There is no erythema or desquamation of the peripheral extremities in scarlet fever, and the patient exhibited no rise in ASOand failed to respond to antibiotics. Twenty-two cases of adult Kawasaki disease have been reported (3-20); Table 2 shows their clinical features. The illness occurred in young adults with a mean age of 24.3 and a male-to-female ratio of 1:1. Butler et al reported a mean age of 25.6 yr and a male-to-female ratio of 2.7:1 in ll adult Kawasaki disease patients (19). Arthralgia was observed in ten cases (45.5%), whereas it is found in 30% of childhood cases (2). Gastrointestinal complications, such as diarrhea, abdominal pain, nausea and vomiting, were reported in 15 cases (68.2%), whereas they occur in 25% of childhood cases (23). Cardiac complications, such as coronary aneurysms and cardiac failure were observed in three cases (13.6%), whereas such complications are found in Number Age/ Arthralgia Gastr - Cardiac Liver ANA Therapy Reference gex enteritis abnormalities dysfunction number 1 18/F NR /F NR /F NR /F /M NR /F NR Asp /M /M ± Asp /M Asp /F ll 18/F NR PSL /M NR Asp /M NR /M NR /M PSL /M Asp /M Asp /F NR Asp /M NR /F /F /F Asp present case ANA, Anti-nuclear antibody; NR, not reported; Asp, aspirin; PSL, prednisolone 287

4 Tomiyama et al 20% of childhood cases (2). Mcllroy et al reported that six out of nine adult Kawasaki disease patients had a documented cardiac abnormality but that none of these patients had coronary aneurysmsor myocardial infarctions (20). Hepatic dysfunction with or without jaundice was detected in 14 cases (63.6%), however, this is seldom seen in childhood cases (2). Attempts to detect ANAwere made in 12 cases with positive results in two cases and ± in one case; this has not been observed in childhood cases (2). The etiology of adult Kawasaki disease is unknown. Infectious etiologies have been reported in the form of Epstein-Barr virus (5) and herpes simplex virus (19). In the present case, the patient was ANA-positive, and her IgE levels were elevated, yet there was no elevation in viral titers, suggesting immunoregulatory mechanisms. Hicks et al reported an adult patient with Kawasaki disease who had an ANAtiter of 1:160 and was positive for cryoglobulins and circulating immunecomplexesand suggested that Kawasaki disease is a manifestation of immunological mechanisms (14). In this series, eight cases were treated with aspirin, two cases were treated with prednisolone and twelve cases received no treatment. All of the patients improved considerably, and none of the patients died, suggesting a good prognosis for adults with Kawasaki disease. If Kawasaki disease is suspected in an adult, aspirin should be started in a dose of mg/kg/day, and on the fourteenth day, if the patient is afebrile, the dose should be reduced to 3-5 mg/kg/day (2). Twodimensional echocardiography should be performed during the third wk after the onset of illness (24). This test should be repeated one month later, and if there are no abnormalities and the sedimentation rate and platelet count are normal, aspirin can be discontinued (2). The number of cases of childhood Kawasaki disease has been increasing every year (25), and there has been a simultaneous increase in reports of Kawasaki disease in adults. Kawasaki disease must not be overlooked in either pediatrics or in the practice of internal medicine in adults. REFERENCES 1) Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. 178, 1967 (in Japanese). Jpn J Allergol 16: 2) Rowley AH, Gonzalez-Crussi F, Shulman ST. Kawasaki syndrome. Rev Infect Dis 10: 1, ) Lee TJ, Vaughan D. Mucocutaneous lymph node syndrome in a young adult. Arch Intern Med 139: 104, ) Everett ED. Mucocutaneous lymph node syndrome (Kawasaki disease) in adults. J Am Med Assoc 242: 542, ) Barbour AG, Krueger GG, Feorino PM, Smith CB. Kawasaki-like disease in a young adult: Association with primary Epstein-Barr virus infection. J AmMed Assoc 241: 397, ) Schlosseberg D, Kandra J, Kreiser J. Possible Kawasaki disease in a 20-year-old woman. Arch Dermatol 115: 1435, ) Glanzer JW, Galbraith WB, Jacobs JP. Kawasaki disease in a 28-year-old man. J AmMed Assoc 244: 1604, ) Watanabe T, Matsui Y, Hino K. Two adult cases of mucocutaneous lymph node syndrome (MCLS). J Jpn Soc Intern Med 69: 1637, 1980 (in Japanese). 9) Milgrom H, Palmer EL, Slovin SF, Morens DM, Freedman SD, Vaughan JH. Kawasaki disease in a healthy young adult. Ann Intern Med 92: 467, ) Takagi S, Oshimi K, Sumiya M, et al. Adult onset mucocutaneous lymph node syndrome with coronary aneurysm. AmHeart J 101: 852, ll) Gomberg R, HammP, Martin A. Mucocutaneous lymph node syndrome (Kawasaki disease) in an adult. West J Med 135: 406, ) Takamoto T, Niwa A, Taniguchi K, Takeuchi J. Acute febrile mucocutaneous lymph node syndrome (Kawasaki disease) in an adult. Clin Cardiol 5: 555, ) Liebmann LI, Mikelic V, Joh MM, Wilson FM. Hydrops of the gallbladder in an adult with Kawasaki disease. J Am Med Assoc 247: 827, ) Hicks JT, Korenyl-Both A, Utsinger PD, Baran EM, McLaughlin GE. Neuromuscular and immunochemical abnormalities in an adult manwith Kawasaki disease. Ann Intern Med 96: 607, ) Marcella JJ, Ursell PC, Goldberger M, Lovejoy W, Fenogilo JJ, Weiss MB. Kawasaki syndrome in an adult: Endomyocardial histology and ventricular function during acute and recovery phases of illness. J AmColl Cardiol 2: 374, ) Burstein F, Metson R, Colman MF, Canalis RF. Kawasaki disease in adults. Arch Otolaryngol 110: 543, ) Kein DE, Geltner JW. Mucocutaneous lymph node syndrome in an adult, with lymph node biopsy correlation. South Med J 78: 872,

5 Kawasaki Disease in Adults Michels TC. Mucocutaneous lymph node syndrome in adults: Differentiation from toxic shock syndrome. Am J Med 80: 724, Butler DF, Hough DR, Friedman SJ, Davis HE. Adult Kawasaki syndrome. Arch Dermatol 123: 1356, Mcllroy MA, Fisher EJ, Saravolatz LD, Hardwicke MB, Wilson FM. Aseptic meningitis complicating adult Kawasaki disease: Case report and review of the literature. Am J Med 87: 106, Kawasaki T, Kosaki F, Okawa S, Shigematu I, YanagawaH. A new infantile acute febrile mucocutaneous lymph node syndrome (MCLS) prevailing in Japan. Pediatrics 54: 271, Margileth AM. Scalded skin syndrome: Diagnosis, differential diagnosis, and management of 42 children. South Med J 68: 447, Melich ME. Kawasaki syndrome (the mucocutaneous lymph node syndrome). Ann Rev Med 33: 569, Hosaki J, Abe S, Yoshimatsu A, Kondo N, Konno S. Observation of coronary arterial lesions in acute febrile mucocutaneous lymph node syndrome (MCLS). Acta Paediatr Jpn 18: 8, Morens DM, Anderson LJ, Hurwitz ES. National surveillance of Kawasaki disease. Pediatrics 65: 21,