A 3-week-old girl presented to the emergency room with respiratory distress
and generalized maculopapular rash. The newborn was hospitalized with a pr
esumptive diagnosis of congenital syphilis, but she died after 2 days of th
erapy. Tissue from the gastrointestinal tract, brain, liver, spleen, and lu
ng was studied by using direct fluorescent antibody and immunohistochemical
analysis (IHC) for Treponema pallidum. The inflammatory infiltrate was cha
racterized by using IHC against CD3, CD20, CD68, and smooth muscle actin. T
he diagnosis of congenital syphilis was confirmed by demonstrating spiroche
tes in tissues with IHC and direct fluorescent antibody examination. IHC sh
owed abundant treponemes in the small intestine and liver and occasional sp
irochetes in the meninges. Bacteria were seen as intact spirochetes, granul
ar staining, or large extracellular collections of antigen. A constant path
ologic feature throughout the tissues was concentric macrophage (CD68-posit
ive) infiltrate around vessels, giving an onion-skin appearance. IHC identi
fied the macrophages as the prime immune response in congenital syphilis.