Etiology And Epidemiology

1. Cause unknown

2. Variation in incidence: 1-10 per 100,000 population

3. Bimodal age: incidence curve with one peak at 15-35 years of age and the other above 50 years of age

4. Sex ratio in children: 3:1 (males to females)

5. Incidence increased among consanguineous family members and among siblings of patients with HD

6. Immunologic disorders: HD is associated with systemic lupus erythematosus (SLE), rheumatoid arthritis, ataxia telangiectasia, and Swiss-type agammaglob-ulinemia

7. Association with Epstein-Barr virus (EBV).

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