Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Kawasaki Disease Clinical Presentation

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Russell W Steele, MD  more...
 
Updated: Sep 13, 2015
 

History

Most children with Kawasaki disease are brought to medical attention because of prolonged fever. Diagnosis of complete cases requires fever of at least 5 days’ duration (though many believe that the diagnosis can be made earlier in otherwise classic presentations). Affected children are usually more irritable than would be expected for the degree of fever. Antibiotic therapy may have been initiated for other diagnoses, but fever persists.

Parents may note that the fever began abruptly. Baker et al found that nonspecific symptoms commonly precede the onset of fever by several days. In order of decreasing frequency, these included the following[14] :

  • Irritability
  • Vomiting
  • Decreased oral intake
  • Cough
  • Diarrhea
  • Rhinorrhea
  • Weakness
  • Abdominal pain
  • Joint pain

Disease phases

The clinical presentation of Kawasaki disease varies over time, with the clinical course conventionally divided into 3 stages: acute, subacute, and convalescent (see the image below). Some authors add a fourth, chronic, phase.

Clinical manifestations and time course of Kawasak Clinical manifestations and time course of Kawasaki disease.

Stage 1: acutefebrilestage

The acute stage begins with an abrupt onset of fever and lasts approximately 7-14 days. The fever is typically high-spiking and remittent, with peak temperatures ranging from 102-104°F (39-40°C) or higher. This fever is not responsive to antibiotics or antipyretics and can persist for up to 3-4 weeks if untreated. With appropriate therapy, high-dose aspirin, and intravenous immunoglobulin (IVIG), the fever typically remits within 48 hours.

In addition to fever, signs and symptoms of this phase may include the following:

  • Irritability
  • Nonexudative bilateral conjunctivitis (90%)
  • Anterior uveitis (70%)
  • Perianal erythema (70%)
  • Erythema and edema on the hands and feet; the latter impedes ambulation
  • Strawberry tongue and lip fissures
  • Hepatic, renal, and GI dysfunction
  • Myocarditis and pericarditis
  • Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm

The mucocutaneous changes and lymphadenopathy are most evident during the acute phase. However, note that erythema and edema of the hands and feet may be the last finding to develop. The diagnosis should be made in this phase.

Stage 2: subacute stage

The subacute stage begins when the fevers have abated, and it continues until week 4-6. The hallmarks of this stage are desquamation of the digits, thrombocytosis (the platelet count may exceed 1 million/μL), and the development of coronary aneurysms. The risk for sudden death is highest at this stage.

Other characteristics of the subacute stage are by persistent irritability, anorexia, and conjunctival injection. Persistence of fever beyond 2-3 weeks may be an indication of recrudescent Kawasaki disease. If fever persists, the outcome is less favorable because of a greater risk of cardiac complications.

Stage 3: convalescent phase

The convalescent phase is marked by complete resolution of clinical signs of the illness, usually within 3 months of presentation. This stage begins with the return to baseline of the acute phase reactants (eg, erythrocyte sedimentation rate, C-reactive protein) and other laboratory abnormalities. During this stage, most of the clinical findings resolve; however, deep transverse grooves across the nails (Beau lines) may become apparent 1-2 months after the onset of fever.

During the convalescent stage, cardiac abnormalities may still be apparent. Smaller coronary artery aneurysms tend to resolve on their own (60% of cases), but larger aneurysms may expand, and myocardial infarction may occur. In patients whose echocardiograms were previously normal, however, detection of new aneurysms is unusual after week 8 of the illness.

Chronic phase

This stage is of clinical importance only in patients who have developed cardiac complications. Its duration is of lifetime significance because an aneurysm formed in childhood may rupture in adulthood. In some cases of aneurysms rupturing in adult life, careful reviews of past medical histories have revealed febrile childhood illnesses of unknown etiology.

Next

Physical Examination

Because no specific test can be performed for Kawasaki disease and no clinical feature is pathognomonic, the diagnosis of Kawasaki disease is based on the presence of a constellation of clinical findings.[1, 2, 3, 4, 5] The diagnostic criteria established by the American Heart Association (AHA) include fever lasting longer than 5 days (fever is an absolute criterion) and 4 of the 5 main clinical features, after diseases with similar findings have been excluded. The 5 major clinical findings are as follows:

  • Changes in the peripheral extremities: Initial reddening or edema of the palms and soles, followed by membranous desquamation of the finger and toe tips or transverse grooves across the fingernails and toenails (Beau lines)
  • Polymorphous rash (not vesicular): Usually generalized but may be limited to the groin or lower extremities
  • Oropharyngeal changes: Erythema, fissuring, and crusting of the lips; strawberry tongue; diffuse mucosal injection of the oropharynx
  • Bilateral, nonexudative, painless bulbar conjunctival injection
  • Acute nonpurulent cervical lymphadenopathy with lymph node diameter greater than 1.5 cm, usually unilateral

The most recent version of the AHA guidelines suggest that if a patient presents with 4 or more of the principal criteria, Kawasaki disease can be diagnosed on day 4 of the fever. Experienced clinicians who have treated many Kawasaki disease patients may establish diagnosis before day 4. Patients who present with coronary artery disease can be diagnosed with Kawasaki disease if they have at least 3 of the 5 major diagnostic criteria.[66]

Kawasaki disease is a diagnosis of exclusion, so other diseases with similar findings (see Differentials) must first be ruled out. Patients with fever who appear to have Kawasaki disease but do not meet diagnostic criteria and have no other identified cause of their illness are said to have atypical or incomplete Kawasaki disease. Laboratory testing supports the diagnosis in these cases (see Workup).

In published cases, 10-45% of patients have incomplete or atypical clinical presentations. The 2 findings that are most commonly absent are cervical lymphadenopathy and polymorphous rash. Cervical lymphadenopathy is the least common of all clinical features, occurring in approximately 40% of cases in the United States; it is more common in Asia. Least likely to be absent are mucous membrane changes, which occur in more than 90% of patients with either typical or atypical forms of the disease.

Japanese researchers have reported a severe form of Kawasaki disease presenting as only fever and cervical lymphadenopathy. These patients are at increased risk of nonresponse to IVIG therapy or development of a coronary artery abnormality.[67]

The erythema, edema, and desquamation in the extremities may limit movement and cause the child to refuse to bear weight. Desquamation of the fingers and toes begins in the periungual region, may involve the palms and soles, and is usually observed 1-2 weeks after the onset of fever. Occurrence is approximately 75%. (See the image below of peeling and erythema.)

Peeling and erythema of the fingertips. Peeling and erythema of the fingertips.

Cervical lymphadenopathy is usually greater than 1.5 cm and unilateral. Changes in the lips and oral cavity include pharyngeal erythema, dry/fissured or swollen lips, and strawberry tongue (occurrence is approximately 90%). The tongue is described as a strawberry tongue because of the diffuse erythema and prominent papillae. (See the images below of related oral manifestations.)

Pediatrics, Kawasaki disease. Note the appearance Pediatrics, Kawasaki disease. Note the appearance of the hand and lips. Photo courtesy of Sam Richardson, MD.
Strawberry tongue. Strawberry tongue.
Oral manifestations of Kawasaki disease: red lips Oral manifestations of Kawasaki disease: red lips and strawberry tongue.

Other findings

Central nervous system findings are reported. Nearly all children with Kawasaki disease are extremely irritable, 33% can present with lethargy, and the neck may be stiff enough to raise suspicion of meningitis. Aseptic meningitis (ie, with cerebrospinal fluid pleocytosis) may be present in half of all patients. Less frequently (and often a temporary finding), patients develop unilateral facial palsy or high-frequency sensorineural hearing loss. Facial palsy has been reported.

Gastrointestinal system findings include diarrhea, vomiting, and abdominal pain in 33% of patients. Rarely, hepatic enlargement and jaundice may occur.

Musculoskeletal system findings occur early in the illness, with patients experiencing diffuse arthralgia that involves multiple joints, both large and small. In the subacute phase, 20-40% of patients present with arthralgia or arthritis, both of which tend to favor the large weight-bearing joints.

Erythema and induration at the site of bacillus Calmette-Guérin (BCG) inoculation is commonly observed in Japan. This is rare in the United States.

In addition to the classic mucocutaneous signs, unusual dermatologic manifestations may include the following[68] :

  • Pustules
  • Erythema multiforme–like lesions
  • Perianal desquamation
  • Macules
  • Papules
  • Measleslike rash
  • Scarlet fever–like rash
  • Pustulovesicular skin eruption
  • Pustular rash

Other clinical features of the disease may include the following:

  • Dehydration from fever and anorexia
  • Pneumonitis
  • Otitis media
  • Orchitis
  • Meatitis, vulvitis, urethritis
  • Uveitis
  • Peripheral extremity gangrene (extremely rare)

A study that reported on the role of respiratory viruses in the pathogenesis of Kawasaki disease found that more than 40% of children with Kawasaki disease also tested positive for respiratory viruses when they were admitted to the hospital. As a result the authors note that a positive respiratory viral PCR or presence of respiratory symptoms at the time of presentation should not be used to exclude a diagnosis of Kawasaki disease.[69, 70]

For more information on features of this illness, see the Medscape Reference articles Dermatologic Manifestations of Kawasaki Disease and Ophthalmologic Manifestations of Kawasaki Disease.

Previous
 
 
Contributor Information and Disclosures
Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Coauthor(s)

Steven J Parrillo, DO, FACOEP, FACEP Clinical Adjunct Professor, Medical Director and Faculty, Disaster Medicine and Management Masters Program, Philadelphia University College of Health Sciences; Associate Professor, Clinical and Educational Scholarship Track, Jefferson Medical College of Thomas Jefferson University; Director, Division of EMS and Disaster Medicine, Albert Einstein Healthcare Network

Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, World Association for Disaster and Emergency Medicine

Disclosure: Nothing to disclose.

Paul R Ogershok, MD Allergist, Allergy, Asthma, and Immunology Clinic, Southwest Regional Medical Center

Paul R Ogershok, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, Pennsylvania Medical Society, West Virginia State Medical Association

Disclosure: Nothing to disclose.

Elena L Jones, MD Clinical Assistant Professor of Dermatology, Columbia University College of Physicians and Surgeons; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center

Disclosure: Nothing to disclose.

Chief Editor

Russell W Steele, MD Clinical Professor, Tulane University School of Medicine; Staff Physician, Ochsner Clinic Foundation

Russell W Steele, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Microbiology, Infectious Diseases Society of America, Louisiana State Medical Society, Pediatric Infectious Diseases Society, Society for Pediatric Research, Southern Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Jeffrey Glenn Bowman, MD, MS Consulting Staff, Highfield MRI, Columbus, Ohio

Disclosure: Nothing to disclose.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

Herbert S Diamond, MD Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Joseph Domachowske, MD Professor of Pediatrics, Microbiology and Immunology, Department of Pediatrics, Division of Infectious Diseases, State University of New York Upstate Medical University

Joseph Domachowske, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society for Microbiology, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Catherine V Parrillo, DO, FACOP, FAAP, Retired, Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine

Catherine V Parrillo, DO, FACOP, FAAP, is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

Martin Weisse, MD Program Director, Associate Professor, Department of Pediatrics, West Virginia University

Martin Weisse, MD is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Grace M Young, MD Associate Professor, Department of Pediatrics, University of Maryland Medical Center

Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians

Disclosure: Nothing to disclose.

References
  1. Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi. 1967 Mar. 16(3):178-222. [Medline].

  2. Wolff AE, Hansen KE, Zakowski L. Acute Kawasaki disease: not just for kids. J Gen Intern Med. 2007 May. 22(5):681-4. [Medline]. [Full Text].

  3. Han RK, Sinclair B, Newman A, Silverman ED, Taylor GW, Walsh P, et al. Recognition and management of Kawasaki disease. CMAJ. 2000 Mar 21. 162(6):807-12. [Medline]. [Full Text].

  4. Gedalia A. Kawasaki disease: 40 years after the original report. Curr Rheumatol Rep. 2007 Aug. 9(4):336-41. [Medline].

  5. Satou GM, Giamelli J, Gewitz MH. Kawasaki disease: diagnosis, management, and long-term implications. Cardiol Rev. 2007 Jul-Aug. 15(4):163-9. [Medline].

  6. Henderson D. Kawasaki Disease Diagnosed by Urine Proteins? Medscape Medical News. December 26, 2012. Available at http://www.medscape.com/viewarticle/776728. Accessed: January 8, 2013.

  7. Kentsis A, Shulman A, Ahmed S, Brennan E, Monuteaux MC et al. Urine proteomics for discovery of improved diagnostic markers of Kawasaki disease. EMBO Mol Med. 2012 Dec 20. [Medline].

  8. Wood L, Tulloh R. Kawasaki disease: diagnosis, management and cardiac sequelae. Expert Rev Cardiovasc Ther. 2007 May. 5(3):553-61. [Medline].

  9. Newburger JW, Takahashi M, Burns JC, Beiser AS, Chung KJ, Duffy CE, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med. 1986 Aug 7. 315(6):341-7. [Medline].

  10. Salguero JS, Durán DG, Peracaula CS, Iznardi CR, Tardío JO. [Refractory Kawasaki disease with coronary aneurysms treated with infliximab]. An Pediatr (Barc). 2010 Nov. 73(5):268-71. [Medline].

  11. Kawamura Y, Kanai T, Takeshita S, Nonoyama S. Use of the urinary trypsin inhibitor ulinastatin for acute Kawasaki disease. Nihon Rinsho. 2014 Sep. 72(9):1650-3. [Medline].

  12. Melish ME, Hicks RM, Larson EJ. Mucocutaneous lymph node syndrome in the United States. Am J Dis Child. 1976 Jun. 130(6):599-607. [Medline].

  13. Newburger JW, Taubert KA, Shulman ST, Rowley AH, Gewitz MH, Takahashi M, et al. Summary and abstracts of the Seventh International Kawasaki Disease Symposium: December 4-7, 2001, Hakone, Japan. Pediatr Res. 2003 Jan. 53(1):153-7. [Medline].

  14. Baker AL, Lu M, Minich LL, Atz AM, Klein GL, Korsin R, et al. Associated symptoms in the ten days before diagnosis of Kawasaki disease. J Pediatr. 2009 Apr. 154(4):592-595.e2. [Medline]. [Full Text].

  15. Rizk SR, El Said G, Daniels LB, Burns JC, El Said H, Sorour KA, et al. Acute Myocardial Ischemia in Adults Secondary to Missed Kawasaki Disease in Childhood. Am J Cardiol. 2014 Nov 29. [Medline].

  16. Son MB, Gauvreau K, Ma L, Baker AL, Sundel RP, Fulton DR, et al. Treatment of Kawasaki disease: analysis of 27 US pediatric hospitals from 2001 to 2006. Pediatrics. 2009 Jul. 124(1):1-8. [Medline].

  17. Rowley AH, Shulman ST. Pathogenesis and management of Kawasaki disease. Expert Rev Anti Infect Ther. 2010 Feb. 8(2):197-203. [Medline]. [Full Text].

  18. Burns JC, Shimizu C, Shike H, Newburger JW, Sundel RP, Baker AL, et al. Family-based association analysis implicates IL-4 in susceptibility to Kawasaki disease. Genes Immun. 2005 Aug. 6(5):438-44. [Medline]. [Full Text].

  19. Lee TJ, Chun JK, Yeon SI, Shin JS, Kim DS. Increased serum levels of macrophage migration inhibitory factor in patients with Kawasaki disease. Scand J Rheumatol. 2007 May-Jun. 36(3):222-5. [Medline].

  20. Leung DY, Schlievert PM, Meissner HC. The immunopathogenesis and management of Kawasaki syndrome. Arthritis Rheum. 1998 Sep. 41(9):1538-47. [Medline].

  21. Pietra BA, De Inocencio J, Giannini EH, Hirsch R. TCR V beta family repertoire and T cell activation markers in Kawasaki disease. J Immunol. 1994 Aug 15. 153(4):1881-8. [Medline].

  22. Wang CL, Wu YT, Liu CA, Kuo HC, Yang KD. Kawasaki disease: infection, immunity and genetics. Pediatr Infect Dis J. 2005 Nov. 24(11):998-1004. [Medline].

  23. Ogawa S. Biomarker. Nihon Rinsho. 2014 Sep. 72(9):1578-84. [Medline].

  24. Onouchi Y. Susceptibility genes for Kawasaki disease. Nihon Rinsho. 2014 Sep. 72(9):1554-60. [Medline].

  25. Hua W, Izurieta HS, Slade B, Belay ED, Haber P, Tiernan R, et al. Kawasaki disease after vaccination: reports to the vaccine adverse event reporting system 1990-2007. Pediatr Infect Dis J. 2009 Nov. 28(11):943-7. [Medline].

  26. Miron D, Fink D, Hashkes PJ. Kawasaki disease in an infant following immunisation with hepatitis B vaccine. Clin Rheumatol. 2003 Dec. 22(6):461-3. [Medline].

  27. Treadwell TA, Maddox RA, Holman RC, Belay ED, Shahriari A, Anderson MS, et al. Investigation of Kawasaki syndrome risk factors in Colorado. Pediatr Infect Dis J. 2002 Oct. 21(10):976-8. [Medline].

  28. Burns JC, Glodé MP. Kawasaki syndrome. Lancet. 2004 Aug 7-13. 364(9433):533-44. [Medline].

  29. Melish ME, Hicks RV. Kawasaki syndrome: clinical features. Pathophysiology, etiology and therapy. J Rheumatol Suppl. 1990 Sep. 24:2-10. [Medline].

  30. Leung DY, Meissner HC, Fulton DR, Murray DL, Kotzin BL, Schlievert PM. Toxic shock syndrome toxin-secreting Staphylococcus aureus in Kawasaki syndrome. Lancet. 1993 Dec 4. 342(8884):1385-8. [Medline].

  31. Yanagawa H, Nakamura Y, Yashiro M, Ojima T, Tanihara S, Oki I, et al. Results of the nationwide epidemiologic survey of Kawasaki disease in 1995 and 1996 in Japan. Pediatrics. 1998 Dec. 102(6):E65. [Medline].

  32. Yanagawa H, Yashiro M, Nakamura Y, Kawasaki T, Kato H. Epidemiologic pictures of Kawasaki disease in Japan: from the nationwide incidence survey in 1991 and 1992. Pediatrics. 1995 Apr. 95(4):475-9. [Medline].

  33. Pinna GS, Kafetzis DA, Tselkas OI, Skevaki CL. Kawasaki disease: an overview. Curr Opin Infect Dis. 2008 Jun. 21(3):263-70. [Medline].

  34. Caquard M, Parlier G, Siret D. [Family observation of Kawasaki disease: 2 cases in sister and brother]. Arch Pediatr. 2006 May. 13(5):453-5. [Medline].

  35. Burns JC, Shimizu C, Gonzalez E, Kulkarni H, Patel S, Shike H, et al. Genetic variations in the receptor-ligand pair CCR5 and CCL3L1 are important determinants of susceptibility to Kawasaki disease. J Infect Dis. 2005 Jul 15. 192(2):344-9. [Medline]. [Full Text].

  36. Uehara R, Yashiro M, Nakamura Y, Yanagawa H. Clinical features of patients with Kawasaki disease whose parents had the same disease. Arch Pediatr Adolesc Med. 2004 Dec. 158(12):1166-9. [Medline].

  37. Mason WH, Takahashi M, Schneider T. Recurrence of Kawasaki disease in a large urban cohort in the United States. In: Takahashi M, Taubert K, eds. Proceedings of the Fourth International Symposium on Kawasaki Disease. Dallas, Tex: American Heart Association; 1993:21-6.:

  38. Kato S, Kimura M, Tsuji K, Kusakawa S, Asai T, Juji T, et al. HLA antigens in Kawasaki disease. Pediatrics. 1978 Feb. 61(2):252-5. [Medline].

  39. Matsuda I, Hattori S, Nagata N, Fruse A, Nambu H. HLA antigens in mucocutaneous lymph node syndrome. Am J Dis Child. 1977 Dec. 131(12):1417-8. [Medline].

  40. Onouchi Y, Tamari M, Takahashi A, Tsunoda T, Yashiro M, Nakamura Y, et al. A genomewide linkage analysis of Kawasaki disease: evidence for linkage to chromosome 12. J Hum Genet. 2007. 52(2):179-90. [Medline].

  41. Dergun M, Kao A, Hauger SB, Newburger JW, Burns JC. Familial occurrence of Kawasaki syndrome in North America. Arch Pediatr Adolesc Med. 2005 Sep. 159(9):876-81. [Medline]. [Full Text].

  42. Burns JC, Cayan DR, Tong G, Bainto EV, Turner CL, Shike H, et al. Seasonality and temporal clustering of Kawasaki syndrome. Epidemiology. 2005 Mar. 16(2):220-5. [Medline]. [Full Text].

  43. Onouchi Y, Gunji T, Burns JC, Shimizu C, Newburger JW, Yashiro M, et al. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms. Nat Genet. 2008 Jan. 40(1):35-42. [Medline]. [Full Text].

  44. Breunis WB, Biezeveld MH, Geissler J, Kuipers IM, Lam J, Ottenkamp J, et al. Polymorphisms in chemokine receptor genes and susceptibility to Kawasaki disease. Clin Exp Immunol. 2007 Oct. 150(1):83-90. [Medline]. [Full Text].

  45. Mamtani M, Matsubara T, Shimizu C, et al. Association of CCR2-CCR5 haplotypes and CCL3L1 copy number with Kawasaki Disease, coronary artery lesions, and IVIG responses in Japanese children. PLoS One. Jul 7 2010;5(7):e11458:

  46. Taniuchi S, Masuda M, Teraguchi M, Ikemoto Y, Komiyama Y, Takahashi H, et al. Polymorphism of Fc gamma RIIa may affect the efficacy of gamma-globulin therapy in Kawasaki disease. J Clin Immunol. 2005 Jul. 25(4):309-13. [Medline].

  47. Holman RC, Belay ED, Christensen KY, Folkema AM, Steiner CA, Schonberger LB. Hospitalizations for Kawasaki syndrome among children in the United States, 1997-2007. Pediatr Infect Dis J. 2010 Jun. 29(6):483-8. [Medline].

  48. Falcini F, Ozen S, Magni-Manzoni S, Candelli M, Ricci L, Martini G, et al. Discrimination between incomplete and atypical Kawasaki syndrome versus other febrile diseases in childhood: results from an international registry-based study. Clin Exp Rheumatol. 2012 Sep-Oct. 30:799-804. [Medline].

  49. Burns JC, Mason WH, Hauger SB, Janai H, Bastian JF, Wohrley JD, et al. Infliximab treatment for refractory Kawasaki syndrome. J Pediatr. 2005 May. 146(5):662-7. [Medline].

  50. Yanagawa H, Nakamura Y, Yashiro M, Uehara R, Oki I, Kayaba K. Incidence of Kawasaki disease in Japan: the nationwide surveys of 1999-2002. Pediatr Int. 2006 Aug. 48(4):356-61. [Medline].

  51. Park YW, Han JW, Park IS, Kim CH, Cha SH, Ma JS, et al. Kawasaki disease in Korea, 2003-2005. Pediatr Infect Dis J. 2007 Sep. 26(9):821-3. [Medline].

  52. Huang GY, Ma XJ, Huang M, Chen SB, Huang MR, Gui YH, et al. Epidemiologic pictures of Kawasaki disease in Shanghai from 1998 through 2002. J Epidemiol. 2006 Jan. 16(1):9-14. [Medline].

  53. Royle JA, Williams K, Elliott E, Sholler G, Nolan T, Allen R, et al. Kawasaki disease in Australia, 1993-95. Arch Dis Child. 1998 Jan. 78(1):33-9. [Medline]. [Full Text].

  54. Harnden A, Alves B, Sheikh A. Rising incidence of Kawasaki disease in England: analysis of hospital admission data. BMJ. 2002 Jun 15. 324(7351):1424-5. [Medline]. [Full Text].

  55. Lin YT, Manlhiot C, Ching JC, Han RK, Nield LE, Dillenburg R, et al. Repeated systematic surveillance of Kawasaki disease in Ontario from 1995 to 2006. Pediatr Int. 2010 Oct. 52(5):699-706. [Medline].

  56. Nakamura Y. Epidemiology of Kawasaki disease. Nihon Rinsho. 2014 Sep. 72(9):1536-41. [Medline].

  57. Stankovic K, Miailhes P, Bessis D, Ferry T, Broussolle C, Sève P. Kawasaki-like syndromes in HIV-infected adults. J Infect. 2007 Dec. 55(6):488-94. [Medline].

  58. Pannaraj PS, Turner CL, Bastian JF, Burns JC. Failure to diagnose Kawasaki disease at the extremes of the pediatric age range. Pediatr Infect Dis J. 2004 Aug. 23(8):789-91. [Medline].

  59. Manlhiot C, Yeung RS, Clarizia NA, Chahal N, McCrindle BW. Kawasaki disease at the extremes of the age spectrum. Pediatrics. 2009 Sep. 124(3):e410-5. [Medline].

  60. Kim T, Choi W, Woo CW, Choi B, Lee J, Lee K, et al. Predictive risk factors for coronary artery abnormalities in Kawasaki disease. Eur J Pediatr. 2007 May. 166(5):421-5. [Medline].

  61. Choi JY, Park SY, Choi KH, Park YH, Lee YH. Clinical characteristics of Kawasaki disease with sterile pyuria. Korean J Pediatr. 2013 Jan. 56(1):13-8. [Medline]. [Full Text].

  62. Gupta-Malhotra M, Gruber D, Abraham SS, Roman MJ, Zabriskie JB, Hudgins LC, et al. Atherosclerosis in survivors of Kawasaki disease. J Pediatr. 2009 Oct. 155(4):572-7. [Medline].

  63. Kitamura S, Tsuda E, Kobayashi J, Nakajima H, Yoshikawa Y, Yagihara T, et al. Twenty-five-year outcome of pediatric coronary artery bypass surgery for Kawasaki disease. Circulation. 2009 Jul 7. 120(1):60-8. [Medline].

  64. Yeo Y, Kim T, Ha K, Jang G, Lee J, Lee K, et al. Incomplete Kawasaki disease in patients younger than 1 year of age: a possible inherent risk factor. Eur J Pediatr. 2009 Feb. 168(2):157-62. [Medline].

  65. Wilder MS, Palinkas LA, Kao AS, Bastian JF, Turner CL, Burns JC. Delayed diagnosis by physicians contributes to the development of coronary artery aneurysms in children with Kawasaki syndrome. Pediatr Infect Dis J. 2007 Mar. 26(3):256-60. [Medline]. [Full Text].

  66. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 2004 Oct 26. 110(17):2747-71. [Medline].

  67. Nomura Y, Arata M, Koriyama C, Masuda K, Morita Y, Hazeki D, et al. A severe form of Kawasaki disease presenting with only fever and cervical lymphadenopathy at admission. J Pediatr. 2010 May. 156(5):786-91. [Medline].

  68. Ulloa-Gutierrez R, Acón-Rojas F, Camacho-Badilla K, Soriano-Fallas A. Pustular rash in Kawasaki syndrome. Pediatr Infect Dis J. 2007 Dec. 26(12):1163-5. [Medline].

  69. Turnier JL, Anderson MS, Heizer HR, Jone PN, Glodé MP, Dominguez SR. Concurrent Respiratory Viruses and Kawasaki Disease. Pediatrics. 2015 Aug 24. [Medline].

  70. Boggs W. Concurrent Respiratory Viruses Common in Children With Kawasaki Disease. http://www.medscape.com/viewarticle/850157. Available at http://www.medscape.com/viewarticle/850157. August 31, 2015; Accessed: September 14, 2015.

  71. Belay ED, Maddox RA, Holman RC, Curns AT, Ballah K, Schonberger LB. Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994-2003. Pediatr Infect Dis J. 2006 Mar. 25(3):245-9. [Medline].

  72. Goo HW, Park IS, Ko JK, Kim YH. Coronary CT angiography and MR angiography of Kawasaki disease. Pediatr Radiol. 2006 Jul. 36(7):697-705. [Medline].

  73. Mavrogeni S, Papadopoulos G, Douskou M, Kaklis S, Seimenis I, Baras P, et al. Magnetic resonance angiography is equivalent to X-ray coronary angiography for the evaluation of coronary arteries in Kawasaki disease. J Am Coll Cardiol. 2004 Feb 18. 43(4):649-52. [Medline].

  74. Newburger JW, Fulton DR. Kawasaki disease. Curr Opin Pediatr. 2004 Oct. 16(5):508-14. [Medline].

  75. Manlhiot C, Christie E, McCrindle BW, Rosenberg H, Chahal N, Yeung RS. Complete and incomplete Kawasaki disease: two sides of the same coin. Eur J Pediatr. 2011 Dec 3. [Medline].

  76. Heuclin T, Dubos F, Hue V, Godart F, Francart C, Vincent P, et al. Increased detection rate of Kawasaki disease using new diagnostic algorithm, including early use of echocardiography. J Pediatr. 2009 Nov. 155(5):695-9.e1. [Medline].

  77. Hinze CH, Graham TB, Sutherell JS. Kawasaki disease without fever. Pediatr Infect Dis J. 2009 Oct. 28(10):927-8. [Medline].

  78. Printz BF, Sleeper LA, Newburger JW, Minich LL, Bradley T, Cohen MS, et al. Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease. J Am Coll Cardiol. 2011 Jan 4. 57(1):86-92. [Medline].

  79. Kanamaru H, Sato Y, Takayama T, Ayusawa M, Karasawa K, Sumitomo N, et al. Assessment of coronary artery abnormalities by multislice spiral computed tomography in adolescents and young adults with Kawasaki disease. Am J Cardiol. 2005 Feb 15. 95(4):522-5. [Medline].

  80. Dadlani GH, Gingell RL, Orie JD, Roland JM, Najdzionek J, Lipsitz SR, et al. Coronary artery calcifications in the long-term follow-up of Kawasaki disease. Am Heart J. 2005 Nov. 150(5):1016. [Medline].

  81. Weedon R. Kawasaki Syndrome: The vasculopathic reaction pattern In:. Pathology. 2002:238-9:

  82. Chen X, Zhao ZW, Li L, Chen XJ, Xu H, Lou JT, et al. Hypercoagulation and elevation of blood triglycerides are characteristics of Kawasaki disease. Lipids Health Dis. 2015 Dec 30. 14(1):166. [Medline].

  83. Watanabe K, Suzuki H, Jiang M, Haniu H, Numano F, Hoshina S, et al. Soluble LR11 is a novel biomarker for vascular lesions late after Kawasaki disease. Atherosclerosis. 2015 Dec 29. 246:94-97. [Medline].

  84. Uehara R, Yashiro M, Oki I, Nakamura Y, Yanagawa H. Re-treatment regimens for acute stage of Kawasaki disease patients who failed to respond to initial intravenous immunoglobulin therapy: analysis from the 17th nationwide survey. Pediatr Int. 2007 Aug. 49(4):427-30. [Medline].

  85. Tremoulet AH, Best BM, Song S, Wang S, Corinaldesi E, Eichenfield JR, et al. Resistance to intravenous immunoglobulin in children with Kawasaki disease. J Pediatr. 2008 Jul. 153(1):117-21. [Medline]. [Full Text].

  86. Sittiwangkul R, Pongprot Y, Silvilairat S, Phornphutkul C. Management and outcome of intravenous gammaglobulin-resistant Kawasaki disease. Singapore Med J. 2006 Sep. 47(9):780-4. [Medline].

  87. Zulian F, Zanon G, Martini G, Mescoli G, Milanesi O. Efficacy of infliximab in long-lasting refractory Kawasaki disease. Clin Exp Rheumatol. 2006 Jul-Aug. 24(4):453. [Medline].

  88. Stenbøg EV, Windelborg B, Hørlyck A, Herlin T. The effect of TNFalpha blockade in complicated, refractory Kawasaki disease. Scand J Rheumatol. 2006 Jul-Aug. 35(4):318-21. [Medline].

  89. Burns JC, Best BM, Mejias A, Mahony L, Fixler DE, Jafri HS, et al. Infliximab treatment of intravenous immunoglobulin-resistant Kawasaki disease. J Pediatr. 2008 Dec. 153(6):833-8. [Medline]. [Full Text].

  90. Youn Y, Kim J, Hong YM, Sohn S. Infliximab as the First Retreatment in Patients with Kawasaki Disease Resistant to Initial Intravenous Immunoglobulin. Pediatr Infect Dis J. 2015 Dec 15. [Medline].

  91. Hsieh KS, Weng KP, Lin CC, Huang TC, Lee CL, Huang SM. Treatment of acute Kawasaki disease: aspirin's role in the febrile stage revisited. Pediatrics. 2004 Dec. 114(6):e689-93. [Medline].

  92. Baumer JH, Love SJ, Gupta A, Haines LC, Maconochie I, Dua JS. Salicylate for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev. 2006 Oct 18. CD004175. [Medline].

  93. Inoue Y, Okada Y, Shinohara M, Kobayashi T, Kobayashi T, Tomomasa T, et al. A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome. J Pediatr. 2006 Sep. 149(3):336-341. [Medline].

  94. Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL, et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med. 2007 Feb 15. 356(7):663-75. [Medline].

  95. Athappan G, Gale S, Ponniah T. Corticosteroid therapy for primary treatment of Kawasaki disease - weight of evidence: a meta-analysis and systematic review of the literature. Cardiovasc J Afr. 2009 Jul-Aug. 20(4):233-6. [Medline].

  96. Phillips B. Review: corticosteroids with intravenous immunoglobulin reduced the incidence of coronary artery aneurysm formation in patients with Kawasaki disease. Arch Dis Child Educ Pract Ed. 2013 Feb 7. [Medline].

  97. Douglas D. IVIG Plus Prednisolone Promising in Refractory Kawasaki Disease. Medscape. Mar 18 2013. [Full Text].

  98. Kobayashi T, Kobayashi T, Morikawa A, et al. Efficacy of intravenous immunoglobulin combined with prednisolone following resistance to initial intravenous immunoglobulin treatment of acute Kawasaki disease. J Pediatr. Aug 2013. 163(2):521-526.e1. [Medline].

 
Previous
Next
 
Patchy generalized macular erythema, which is also typical of some viral exanthems.
Peeling and erythema of the fingertips.
Strawberry tongue.
Pediatrics, Kawasaki disease. Note the appearance of the hand and lips. Photo courtesy of Sam Richardson, MD.
Clinical manifestations and time course of Kawasaki disease.
Oral manifestations of Kawasaki disease: red lips and strawberry tongue.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.