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Kawasaki disease is an acute and multisystemic vasculitis that impairs medium-sized vessels, with predilection for the coronary arteries.[1]The causative agent of KD has not been still indentified and the aetiopathogenetic theories are based on epidemiologic, laboratory and histological data.[2] more than 80% of affected children are younger than 5 years of age.[3][4] Although this type of vasculitis can affect many organs,[5] the most important and feared involvement is that of the coronary arteries,[6]it is more common with younger age, and early initiation of the patient management is key to prevent this fatal complication.[7]


The disease was first reported by Dr. Tomisaku Kawasaki in a four-year-old child with a rash and fever at the Red Cross Hospital in Tokyo, Japan in January of 1961, and later published a report on 50 similar cases.[8]Later Yamamoto and colleagues were persuade that there is definite cardiac involvement when they studied and reported 23 cases, of which 11(48%) patients had abnormalities detected by an electrocardiogram.[9] It was not until 1974 that the first description of this disorder was published in the English language literature.[10]in the year 1976 Melish et al., described the same illness in 16 children in Hawaii.[11]Melish and Kawasaki had independently developed the same diagnostic criteria for the disorder, which are still used today to make the diagnosis of classic KS.

A question was raised whether the disease only started during the period between 1960 and 1970, but later a preserved heart of a 7 year old boy died in 1870 was examined and showed three aneurysms of the coronary arteries with clots, as well as pathologic changes consistent with KS.[12] KS is now recognized worldwide. In the United States and other developed nations, it appears to have replaced acute rheumatic fever as the most common cause of acquired heart disease in children.[13]

Clinical Features[edit]

The most prominent symptom in Kawasaki disease is fever, which is a characteristic sign of the acute phase of the disease, is normally high (above 39-40º C), remittent and followed by extreme irritability.[14][15] recent reports says it is even present in patients with atypical or incomplete KD,[16][17]nevertheless recent reports says it is not present on 100% of cases.[18] The first day of fever is considered the first day of illness,[19] and the duration of fever is on average one to two weeks; in the absence of treatment, it may extend for three to four weeks,[20] Prolonged fever is associated with higher incidence of cardiac involvement.[21] It responds partially to antipyretic drugs and does not cease with the introduction of antibiotics.[20] However, when appropriate therapy is started – intravenous immunoglobulin (IVIG) and aspirin – the fever is gone after two days.[22]

Bilateral conjunctival injection was reported by many publications to be the most common symptom after fever,[23][24] it typically involves the bulbar conjunctivae, is not accompanied by suppuration, it is not painful.[25] It usually begins shortly after the onset of fever during the acute stage of the disease.[19] Anterior uveitis may be present on slit-lamp examination.[26][27] iritis can occur too. [28]

KD presents with set of oral manifestations, the most characteristic changes are the bright red, swollen lips with vertical cracking and bleeding.[29] The mucosa of the oropharynx may be bright red, and the tongue may have a typical "strawberry" appearance. [20] These oral manifestations are caused by the typical necrotizing microvasculitis with fibrinoid necrosis.[29]

In the acute phase of the disease changes in the peripheral extremities can include erythema of the palms and soles, which is often striking with sharp demarcation,[19] and often accompanied by painful, brawny edema of the dorsa of the hands or feet in the acute phase of the disease, and it is why affected children frequently refuse to hold objects in their hands or to bear weight on their feet.[19][20] Later during the convalescent or the subacute phase desquamation of the fingers and toes usually begins in the periungual region within two to three weeks after the onset of fever and may extend to include the palms and soles,[30] and around 11% of children affected by the disease may continue skin peeling for many years.[31] 1 to 2 months after the onset of fever deep transverse grooves across the nails may develop (Beau’s lines),[32] And occasionally nail are shed.[32]

The most common cutaneous manifestation is a diffuse maculopapular erythematous rash, which is quite nonspecific.[33] The rash varies over time and is characteristically located on the trunk and may further spread to involve the face, extremities, and perineum.[20] many other forms of cutaneous lesions have been reported also, they may include Scarlatiniform, popular, multiforme, and purpuric lesions, even micropustules were reported,[34][35] however it is never bullous or vesicular[20]

Cervical lymphadenopathy, is seen in approximately 50 to 75% of patients, whereas the other features are estimated to occur in 90% of patients.[19][23] But sometimes it can be the dominant presenting symptom.[36][37] According to the definition of the diagnostic criteria, there should be more than one impaired lymph node and > 1.5 cm in diameter.[38] Affected lymph nodes are not painful or little painful, not-fluctuating and non-suppurative; erythema of the neighboring skin may occur.[19] We should be attentive to those children with fever and neck adenitis that do not respond to antibiotics, because Kawasaki disease should be part of the differential diagnoses.[19]

The course[edit]

The course of the disease can be divided into three clinical phases.[38] The acute febrile phase, which usually lasts for 1 to 2 weeks, is characterized by fever, conjunctival injection, erythema of the oral mucosa, erythema and swelling of the hands and feet, rash, cervical adenopathy, aseptic meningitis, diarrhea, and hepatic dysfunction.[38] Myocarditis is common during this time, and a pericardial effusion may be present.[19] Coronary arteritis may be present, but aneurysms are generally not yet visible by echocardiography. The subacute phase begins when fever, rash, and lymphadenopathy resolve at about 1 to 2 weeks after the onset of fever, but irritability, anorexia, and conjunctival injection persist. Desquamation of the fingers and toes and thrombocytosis are seen during this stage, which generally lasts until about 4 weeks after the onset of fever. Coronary artery aneurysms usually develop during this time, and the risk for sudden death is highest during this stage.[19] The convalescent stage begins when all clinical signs of illness have disappeared and continues until the sedimentation rate returns to normal, usually at 6 to 8 weeks after the onset of illness.[38]

Paper reference[edit]

Associated symptoms of kawasaki disease.[23] Kawasaki disease: A brief history.[39]


  1. ^ Markovich DS, Krapivinskaya LD (1975). "The investigation of regulatory transition in D-glyceraldehyde-3-phosphate dehydrogenase quaternary structure". FEBS Letters. 58 (1): 138–40. PMID 178533.  Unknown parameter |month= ignored (help)
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  5. ^ Amano S, Hazama F, Kubagawa H, Tasaka K, Haebara H, Hamashima Y (1980). "General pathology of Kawasaki disease. On the morphological alterations corresponding to the clinical manifestations". Acta Pathologica Japonica. 30 (5): 681–94. PMID 7446109.  Unknown parameter |month= ignored (help)
  6. ^ Hirose O, Misawa H, Kijima Y; et al. (1981). "[Two-dimensional echocardiography of coronary artery in Kawasaki disease (MCLS): detection, changes in acute phase, and follow-up observation of the aneurysm (author's transl)]". Journal of Cardiography (in Japanese). 11 (1): 89–104. PMID 7264399.  Unknown parameter |month= ignored (help)
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  8. ^ Kawasaki T (1967). "[Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]". [[Arerugī = [Allergy]]] (in Japanese). 16 (3): 178–222. PMID 6062087.  Unknown parameter |month= ignored (help)
  9. ^ Yamamoto T, Oya T, Watanabe A, et al. Clinical features of Kawasaki disease [in Japanese] Shonika Rinsho (Jpn J Pediatr) 1968;21:291–297.
  10. ^ Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H (1974). "A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan". Pediatrics. 54 (3): 271–6. PMID 4153258.  Unknown parameter |month= ignored (help)
  11. ^ Melish ME, Hicks RM, Larson EJ (1976). "Mucocutaneous lymph node syndrome in the United States". American Journal of Diseases of Children (1960). 130 (6): 599–607. PMID 7134.  Unknown parameter |month= ignored (help)
  12. ^ Gee S J. Cases of morbid anatomy. St Bartholomew’s Hosp Rep. 1871;7:141–148.
  13. ^ Taubert K A, Rowley A H, Shulman S T. A 10 year (1984–1993) United States hospital survey of Kawasaki disease. In: Kato H, editor. Kawasaki disease. Amsterdam, The Netherlands: Elsevier Science B. V.; 1995. pp. 34–38.
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  15. ^ Cassidy JT, Petty RE. Vasculitis. In: Cassidy JT, Petty RE, eds. Textbook of pediatric rheumatology. 3rd ed. Philadelphia, W.B: Saunders Company; 1995. p. 365-422
  16. ^ Fukushige J, Takahashi N, Ueda Y, Ueda K (1994). "Incidence and clinical features of incomplete Kawasaki disease". Acta Paediatrica (Oslo, Norway : 1992). 83 (10): 1057–60. PMID 7841704.  Unknown parameter |month= ignored (help)
  17. ^ Rowley AH, Gonzalez-Crussi F, Gidding SS, Duffy CE, Shulman ST (1987). "Incomplete Kawasaki disease with coronary artery involvement". The Journal of Pediatrics. 110 (3): 409–13. PMID 3819942.  Unknown parameter |month= ignored (help)
  18. ^ Rodriguez-Lozano AL, Rivas-Larrauri FE, Hernandez-Bautista VM, Yamazaki-Nakashimada MA (2011). "Fever is not always present in Kawasaki disease". Rheumatology International. doi:10.1007/s00296-011-2123-4. PMID 21881982.  Unknown parameter |month= ignored (help)
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  21. ^ Mori M, Imagawa T, Yasui K, Kanaya A, Yokota S (2000). "Predictors of coronary artery lesions after intravenous gamma-globulin treatment in Kawasaki disease". The Journal of Pediatrics. 137 (2): 177–80. doi:10.1067/mpd.2000.107890. PMID 10931408.  Unknown parameter |month= ignored (help)
  22. ^ Newburger JW, Takahashi M, Beiser AS; et al. (1991). "A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome". The New England Journal of Medicine. 324 (23): 1633–9. doi:10.1056/NEJM199106063242305. PMID 1709446.  Unknown parameter |month= ignored (help)
  23. ^ a b Yun SH, Yang NR, Park SA (2011). "Associated symptoms of kawasaki disease". Korean Circulation Journal. 41 (7): 394–8. doi:10.4070/kcj.2011.41.7.394. PMC 3152734Freely accessible. PMID 21860641.  Unknown parameter |month= ignored (help)
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  28. ^ Smith LB, Newburger JW, Burns JC (1989). "Kawasaki syndrome and the eye". The Pediatric Infectious Disease Journal. 8 (2): 116–8. PMID 2468129.  Unknown parameter |month= ignored (help)
  29. ^ a b Scardina GA, Fucà G, Carini F; et al. (2007). "Oral necrotizing microvasculitis in a patient affected by Kawasaki disease". Medicina Oral, Patología Oral Y Cirugía Bucal. 12 (8): E560–4. PMID 18059239.  Unknown parameter |month= ignored (help)
  30. ^ Wang S, Best BM, Burns JC (2009). "Periungual desquamation in patients with Kawasaki disease". The Pediatric Infectious Disease Journal. 28 (6): 538–9. doi:10.1097/INF.0b013e3181945984. PMC 2738931Freely accessible. PMID 19483521.  Unknown parameter |month= ignored (help)
  31. ^ Michie C, Kinsler V, Tulloh R, Davidson S (2000). "Recurrent skin peeling following Kawasaki disease". Archives of Disease in Childhood. 83 (4): 353–5. PMC 1718513Freely accessible. PMID 10999876.  Unknown parameter |month= ignored (help)
  32. ^ a b López Neyra A, Alvarez-Coca González J, Pérez Suárez E, Martínez Pérez J, Rubio Villanueva JL (2007). "[Beau's lines and Kawasaki disease]". Anales De Pediatría (Barcelona, Spain : 2003) (in Spanish; Castilian). 67 (6): 610–1. PMID 18053534.  Unknown parameter |month= ignored (help)
  33. ^ González Pascual E, Villanueva Lamas J, Ros Viladoms J, Pons Odena M, Ruiz García-Diego S (1999). "[Kawasaki disease. A report of 50 cases]". Anales Españoles De Pediatría (in Spanish; Castilian). 50 (1): 39–43. PMID 10083641.  Unknown parameter |month= ignored (help)
  34. ^ Kwan YW, Leung CW (2005). "Pustulo-vesicular skin eruption in a child with probable Kawasaki disease". European Journal of Pediatrics. 164 (12): 770–1. doi:10.1007/s00431-005-1715-y. PMID 16010565.  Unknown parameter |month= ignored (help)
  35. ^ Ulloa-Gutierrez R, Acón-Rojas F, Camacho-Badilla K, Soriano-Fallas A (2007). "Pustular rash in Kawasaki syndrome". The Pediatric Infectious Disease Journal. 26 (12): 1163–5. doi:10.1097/INF.0b013e31814619ec. PMID 18043462.  Unknown parameter |month= ignored (help)
  36. ^ Stamos JK, Corydon K, Donaldson J, Shulman ST (1994). "Lymphadenitis as the dominant manifestation of Kawasaki disease". Pediatrics. 93 (3): 525–8. PMID 8115224.  Unknown parameter |month= ignored (help)
  37. ^ Kubota M, Usami I, Yamakawa M, Tomita Y, Haruta T (2008). "Kawasaki disease with lymphadenopathy and fever as sole initial manifestations". Journal of Paediatrics and Child Health. 44 (6): 359–62. doi:10.1111/j.1440-1754.2008.01310.x. PMID 18476929.  Unknown parameter |month= ignored (help)
  38. ^ a b c d Castro PA, Urbano LM, Costa IM (2009). "[Kawasaki disease]". Anais Brasileiros De Dermatologia (in Portuguese). 84 (4): 317–29. PMID 19851663.  Unknown parameter |month= ignored (help)
  39. ^ Burns JC, Kushner HI, Bastian JF; et al. (2000). "Kawasaki disease: A brief history". Pediatrics. 106 (2): E27. PMID 10920183.  Unknown parameter |month= ignored (help)