Kawasaki disease

Definition: Kawasaki disease or mucocutaneous lymph node syndrome is an acute exanthematous illness of childhood of unknown etiology with a recent marked increase in incidence.

Kawasaki disease (KD) is an inflammatory condition of unknown etiology. It involves mainly the skin, mucous membranes, lymph nodes, and myocardium. Clinical and epidemiologic features of KS are consistent with an infectious cause. The essential lesion is an arteritis and periarteritis initially involving small arteries and later the medium and large vessels.

Synopsis

 systemic vasculitis

• cardiac vasculitis
  • ruptured coronary artery aneurysm
  • myocardial infarction
  • sudden infant death (11774178)

• vasculitis
  • vascular neutrophilic infiltration (15910456)
  • vascular macrophagic infiltration
  • vascular plasmacytic infiltration (IgA plasma cells)
  • vascular proliferation
  • microthrombi
  • focal necrosis around freshly thrombosed vessels

 myocarditis

• cardiac failure

 pulmonary lesions (12833488)

• pulmonary nodules (12833488)
• predominantly mononuclear cell infiltrates in lung parenchyma and infiltrating vessel walls
• IgA plasma-cell infiltration
• interstitial micronodular infiltrates
• inflammatory pulmonary nodules

 IgA plasma-cell vasculitis (9550392)
 IgA plasma-cell infiltration in the vessel walls, kidneys, pancreas, and upper respiratory tract
 acute tonsillitis (16328404)

 digestive Kawasaki disease (16150324)

• surgical abdomen with suspected small bowel obstruction (16150324)
• intestinal obstruction (8811576, 3829564, 3627858)
  • jejunal obstruction (8811576)
• ischemic small bowel strictures (11283899)

• intestinal pseudoobstruction syndrome (8811576, 15121996, 11355064)
• mesenteric vasculitis (11283899)
• ischemic small bowel strictures (11283899)
• colonic ischemia (ischemic colitis) (3794953)
• massive necrosis of duodenum (7277136)
• massive necrosis of jejunum (7277136)

 gallbladder obstruction (7277136)
 massive necrosis of gallbladder (7277136)
 massive necrosis of liver (7277136)
 necrosis of digits of both hands (7277136)
 necrosis of adrenals (7277136)
 necrosis of testes (7277136)
 necrosis of bladder (7277136)
 necrosis of stomach (7277136)

 acute tonsillitis (16328404)
 hepatitis

• hepatomegaly
• portal acute inflammation

 pancreatic Kawasaki disease

• pancreatic macrophagic infiltration (14688578)

  splenomegaly (3835550)
 nodal Kawasaki disease (lymph node lesions) (7149090)

• multiple foci of necrosis
• fibrin thrombi within the microvasculature

Associations

 meconium ileus equivalent syndrome in cystic fibrosis (1676312)
 parvovirus B19 infection (11864255, 7588963, 7912323, 8466697)
 HIV1 infection (11864255, 8466697)
 transient erythroblastopenia (8775567)
 primary CMV infection (16080076)

Differential diagnosis

 acute adenoviral infection (11864255)

See also

 vasculitites (vasculitis)

References

 Histopathological study of lymph node lesions in the acute phase of Kawasaki disease. Yokouchi Y, Oharaseki T, Harada M, Ihara F, Naoe S, Takahashi K. Histopathology. 2012 Jul 27. doi : 10.1111/his.12007 PMID: 23020240

 Burns JC, Shimizu C, Gonzalez E, Kulkarni H, Patel S, Shike H, Sundel RS, Newburger JW, Ahuja SK. 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. PMID: 15962231

 Maresi E, Passantino R, Midulla R, Ottoveggio G, Orlando E, Becchina G, Meschis L, Amato G. Sudden infant death caused by a ruptured coronary aneurysm during acute phase of atypical Kawasaki disease. Hum Pathol. 2001 Dec;32(12):1407-9. PMID: 11774178

 Terai M, Kohno Y, Namba M, Umemiya T, Niwa K, Nakajima H, Mikata A. Class II major histocompatibility antigen expression on coronary arterial endothelium in a patient with Kawasaki disease. Hum Pathol. 1990 Feb;21(2):231-4. PMID: 2307451