Congenital asplenia presenting as purpura fulminans

We present a case of previously unrecognized congenital absence of the spleen presenting as overwhelming purpura fulminans due to pneumococcal sepsis. This entity is rare but may be recognized incidentally before sepsis develops either by abdominal scanning or by the presence of Howell-Jolly bodies on peripheral blood examination. Clinicians should be aware of the existence of this entity even in the absence of other stigmata of congenital asplenia as may be found in eponymous syndromes (Ivemark, Pearson, and Stormoken). If discovered, patient vaccination and education should be vigorously pursued.