Congenital Syphilis–Persisting Pestilence

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Perinatally acquired syphilis annually affects an estimated 713,600 to 1,575,000 pregnancies worldwide via spontaneous abortion, stillbirth, or congenital syphilis. Sub-Saharan Africa, congenital syphilis has a role in 21% of perinatal deaths.1 In the United States congenital syphilis increased to 10.5 per 100,000 live births in 2007 from a nadir of 8.2 in 2005, where it had fallen from 107.3 per 100,000 in 1991. Rates of primary and secondary syphilis have increased among all age groups in the United States in recent years, with greater increases among males than females.2PATHOGENESISVenereal and congenital syphilis are caused by Treponema pallidum subspecies (Tp-s) pallidum, which has a small genome of 1.14 Mb. Three other subspecies, Tp-s pertenue (yaws), Tp-s carateum (pinta), and Tp-s endemicum (endemic syphilis), are morphologically identical, share high DNA homology, are closely related antigenically to Tp-s pallidum, but are not transmitted perinatally and do not cause neurologic disease.3Multiple variants of the TprK outer membrane protein are encoded by a gene family. The infecting inoculum gives rise to multiple subpopulations with antigenic diversity of expressed TprKs over time. This provides considerable immune response-evasion capacity despite strong humoral and cell-mediated responses that develop soon after infection.4 Eradication occurs when T cells infiltrate syphilitic lesions and activate phagocytosis of antibody-opsonized treponemes by CD68-positive macrophages.5Congenital syphilis arises from transplacental transmission during maternal spirochetemia or during birth by contact with infectious lesions. Transplacental infection occurs as early as 9 to 10 weeks gestation, results in wide dissemination, and is more frequent during primary or secondary syphilis. In the first 4 years after untreated primary infection, risk of transmission is as high as 80% but diminishes thereafter. The infected placenta is usually thickened with villi that are hypercellular with acute and chronic inflammation and proliferative fetal vascular changes. Spirochetes can be found in umbilical vessel walls.2,6,7CLINICAL MANIFESTATIONSTwo-thirds of live born infants are asymptomatic. Fatal neonatal cases are uncommon and usually occur with absent or inadequate maternal treatment. Early signs can be present at birth or appear in the first 2 years of life, while late signs arise duirng the first 2 decades of life.Among 139 infants with symptomatic congenital syphilis in 3 series, the most common early signs were hepatosplenomegaly (71%), rash (68%), fever (42%), neurosyphylis (23%), pneumonitis (17%), rhinitis (snuffles; 14%), generalized lymphadenopathy (14%), and ascites (9%). Leukocytosis occurred in 72%, Coombs-negative hemolytic anemia in 58%, thrombocytopenia in 40%, renal involvement (e.g., proteinuria, hematuria) in 16%, and periostitis or osteochondritis in 78%. Hemolytic anemia may persist for weeks after treatment.8–10Syphilitic hepatitis leads to jaundice and elevated serum transaminases and alkaline phosphatase. Prothrombin time may be prolonged. Skeletal involvement is usually multifocal and symmetric when present. Associated pain with refusal to move involved extremities is pseudoparalysis of Parrot. Demineralization of the upper medial tibial metaphysic is Wimberger sign. Bony involvement usually resolves spontaneously during the first 6 months of life.10Syphilitic pemphigus may be present at birth or develop during the first weeks of life. Vesiculobullous lesions may be preceded by red papules reminiscent of secondary syphilis. Lesions desquamate and crust over in 1–3 wks. Condylomata may arise on mucous membranes or areas of skin affected by moisture. Mucous patches (shallow ulcers with grey exudate and erythematous borders) may develop on oral or genital membranes. Fissures occur around the lips, nares, or anus. Snuffles develop after the first week of life and discharge is often blood-tinged.

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