A previously healthy male infant developed hepatosplenomegaly, severe anaem
ia and thrombocytopenia 5 weeks after birth. Marked haemophagocytosis was p
resent in the bone marrow. A typical maculopapular rash suggested early con
genital syphilis. The diagnosis was confirmed by serology and by the presen
ce of untreated syphilis in both parents.
Conclusion Syphilis needs to be excluded in infants suspected of haemophago
cytic lymphohistiocytosis.