mucocutaneous lymph node syndrome


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Related to mucocutaneous lymph node syndrome: Kawasaki syndrome

mucocutaneous

 [mu″ko-ku-ta´ne-us]
pertaining to mucous membrane and skin.
mucocutaneous lymph node syndrome Kawasaki's disease.

mu·co·cu·ta·ne·ous lymph node syn·drome

a systemic vasculitis of unknown origin that occurs primarily in children under 8 years of age. Symptoms include a fever lasting more than 5 days; polymorphic rash; erythematous, dry, cracking lips; conjunctival injection; swelling of the hands and feet; irritability; adenopathy; and a perineal desquamative rash. Approximately 20% of untreated patients may develop coronary artery aneurysms. As the child recovers from the illness, thrombocytosis and peeling of the fingertips occur.

Kawasaki syndrome

An acute idioapathic febrile illness that primarily affects children under age 5, especially males, and has a winter-spring seasonality.

Clinical findings
Fever; cervical lymphadenopathy; palmoplantar and mucosal erythaema and oedema; conjunctivitis; gingivitis; maculoerythematous glove-and-sock rash over the hands and feet, which becomes hard, oedematous and sloughs; aneurysms of small- and medium-sized coronary, occasionally peripheral, arteries, with arteritis. Other findings include conjunctival oedema, upper respiratory tract oedema, pharyngeal injection and truncal lymphadenopathy.
 
Aetiology
Uncertain; various bacteria have been implicated, though none definitively.
 
Lab
Increased ESR, CRP, complement, immunoglobulins.
 
Management
Gammaglobulin in IV bolus, aspirin IV.
 
Prognosis
May cause sudden death; 1–5% die of disease.

Kawasaki syndrome case definition (CDC)
Fever of 5 days’ duration (or fever post-IV immunoglobulin if given before 5th day of fever), and 4 of following 5 signs:
• Rash;     
• Cervical lymphadenopathy (1.5+ cm in diameter);
• Bilateral conjunctival injection;   
• Oral mucosal changes—erythaema, fissuring and xerostomia, “strawberry tongue”;    
• Peripheral extremity changes—e.g., acral erythema or oedema, periungual and/or generalised desquamation, polymorphous exanthematous rash.

Ka·wa·sa·ki dis·ease

, Kawasaki syndrome (kă-wă-sah'kē di-zēz', sin'drōm)
A systemic vasculitis of unknown origin that occurs primarily in children younger than 8 years of age. Symptoms include a fever lasting more than 5 days; polymorphic rash; erythematous, dry, cracking lips; conjunctival injection; swelling of the hands and feet; irritability; adenopathy; and a perineal desquamative rash. Approximately 20% of untreated patients may develop coronary artery aneurysms. Treatment includes oral aspirin at high dosage, immune globulin administered intravenously, and supportive care. As the child recovers from the illness, thrombocytosis and peeling of the fingertips occur.
Synonym(s): mucocutaneous lymph node syndrome.

Mucocutaneous lymph node syndrome (MLNS)

Mucocutaneous lymph node syndrome, another name for Kawasaki syndrome. The name comes from the key symptoms of the disease, which involve the mucous membranes of the mouth and throat, the skin, and the lymph nodes.
Mentioned in: Kawasaki Syndrome

Kawasaki,

Tomisaku, 20th century Japanese pediatrician.
Kawasaki disease - a polymorphous erythematous febrile, sometimes epidemic, disease of unknown etiology occurring in children. Synonym(s): Kawasaki syndrome; mucocutaneous lymph node syndrome
Kawasaki syndrome - Synonym(s): Kawasaki disease

mu·co·cu·ta·ne·ous lymph node syn·drome

(myū'kō-kyū-tā'nē-ŭs limf nōd sin'drōm)
Systemic vasculitis of unknown origin that occurs primarily in children younger than 8 years of age.
Synonym(s): Kawasaki disease.