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Kawasaki disease

mucocutaneous lymph node syndrome, MCLS

 

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

-  myocarditis

-  pulmonary lesions (#12833488#)

-  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#)

-  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

-  pancreatic Kawasaki disease

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

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#)

References

-  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#



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