eMedicine Specialties > Pediatrics: General Medicine > Infectious Disease

Syphilis

Author: Muhammad Waseem, MD, Associate Professor of Emergency Medicine in Clinical Pediatrics, Weill Medical College of Cornell University; Consulting Staff, Department of Pediatrics, Bronx Lebanon Hospital; Consulting Staff, Department of Emergency Medicine, Lincoln Medical and Mental Health Center
Coauthor(s): Muhammad Aslam, MD, Instructor in Pediatrics, Harvard Medical School; Staff Physician, Department of Medicine/ Division of Newborn Medicine, Children's Hospital Boston
Contributor Information and Disclosures

Updated: Jul 28, 2009

Introduction

Background

Syphilis is a communicable disease caused by Treponema pallidum, which belongs to the Spirochaetaceae family. The genus name, Treponema, is derived from the Greek term for "turning thread." Pathogenic members of this genus include T pallidum, Treponema pertenue, and Treponema carateum.

Between 1905 and 1910, Schaudinn and Hoffman identified T pallidum as the cause of syphilis, and Wasserman described a diagnostic test for the long-recognized infection. Pathogenic treponemes are associated with the following 4 diseases:

• Venereal syphilis, caused by T pallidum pallidum
• Yaws, caused by T pallidum pertenue
• Endemic syphilis (bejel), caused by T pallidum endemicum
• Pinta, caused by T carateum

The treponemes responsible for these diseases cannot be distinguished serologically, morphologically, or by genome analysis, and they have not been successfully cultivated on artificial media.

Pathophysiology

When untreated, syphilis is a lifelong infection that progresses in 3 clear characteristic stages. After initial invasion through mucous membranes or skin, the organism rapidly multiplies and widely disseminates. The organism spreads through the perivascular lymphatics and then the systemic circulation before clinical development of the primary lesion. The primary lesion, which contains infectious treponemes, arises within hours after infection and persists throughout primary and secondary disease.

Secondary lesions develop when spirochetal invasion of tissues of ectodermal origin (eg, skin, mucous membranes, CNS) precipitates an inflammatory response. These lesions develop 6-12 weeks after infection. This stage of rapid spirochete multiplication and dissemination may bring invasion of the entire body. Thus, tertiary syphilis may involve any organ system.

Secondary infection becomes latent within 1-2 months after the rash onset. Relapses with secondary manifestations can be seen during the first year of latency, a period referred to as the early latent period. Early latent syphilis (ie, duration <1 y) is when the recurrent lesions of secondary syphilis are most likely to occur. No relapses occur after the first year; what follows is late syphilis, which may be either asymptomatic (ie, late latent) or symptomatic (ie, tertiary). Late latent syphilis is associated with resistance to both reinfection and relapse.

Tertiary neurosyphilis can manifest in various ways. Meningeal syphilis rarely occurs and presents a few years following the original infection. Late neurosyphilis may present with focal ischemia of the CNS or stroke as a result of endarteritis of small blood vessels of the brain. Meningovascular syphilis can affect any part of the CNS. Actual destruction of the nerve cells in the cerebral cortex leads to a combination of psychiatric manifestations and neurologic findings.

Congenital syphilis is caused by transplacental transmission of spirochetes; the transmission rate approaches 100%. Perinatal death may result from congenital infection in more than 40% of affected, untreated pregnancies. Among survivors, manifestations have traditionally been divided into early and late stages. Manifestations are defined as early if they appear in the first 2 years of life and late if they develop after age 2 years.

Because inflammatory changes do not occur in the fetus until after the first trimester of pregnancy, organogenesis is unaffected. Nevertheless, all organ systems may be involved. With early onset disease, manifestations result from transplacental spirochetemia and are analogous to the secondary stage of acquired syphilis. Congenital syphilis does not have a primary stage. Late-onset disease is seen in patients older than 2 years and is not considered contagious.

Frequency

United States

From 1985-1990, overall syphilis incidence increased 75%. This resurgence was primarily due to increased illegal drug use (particularly crack cocaine) that was associated with an exchange of sex for drugs. Concomitant infection with human immunodeficiency virus (HIV) is also common because HIV and syphilis affect similar patient groups.

An overall increase in syphilis incidence has been observed in the United States, and most infected individuals are men who have sex with other men; this may also lead to an increase in HIV infection and other sexually transmitted diseases (STDs).¹

International

The disease occurs worldwide, predominantly in large cities. With the exception of the United States, syphilis is less common in developed nations.

Certain European countries have seen an increase in congenital syphilis cases, and syphilis remains a major public health problem in sub-Saharan Africa and in the developing world. The main focus in controlling syphilis is antenatal screening and treatment of mothers who are infection.
 
A high prevalence of syphilis and other STDs was noted in Venezuela in a recent study.²

Mortality/Morbidity

Syphilis causes untold morbidity and remains a significant cause of mortality if left untreated.

Race

Syphilis has no racial predilection, although its incidence appears to correlate with the socioeconomic factors that contribute to disease prevalence among individuals with low incomes, who live in urban and overcrowded areas, in whom drug use and the exchange of sex for drugs may be more common.

Sex

Historically, men were more commonly infected than women; however, a study involving high-risk adolescents has reported 69% of cases involved young women, indicating that the sex distribution of syphilis is in flux.

Age

Adolescent and young adults are most at risk due to sexual and other risk-taking behaviors (eg, drug use).

Clinical

History

Most recognized syphilitic disease in children is congenital. A pregnant woman with syphilis who has not received therapy or who has received inadequate therapy may transmit the infection to the fetus at any clinical stage of the disease. Assume children with acquired syphilis have been infected through sexual abuse, unless another method of transmission is identified. Syphilis, previously known as the great imitator, can have numerous and complex manifestations.

• Primary syphilis: The primary lesion, called a chancre, is painless but slightly tender. It usually develops at the site of the infection an average of 3 weeks after exposure to T pallidum. Patients may ignore a visible lesion because it is painless unless it becomes secondarily infected.
• Secondary syphilis
  • Because of the widespread dissemination of spirochetes, frequent constitutional symptoms include fever, malaise, pharyngitis, rash, anorexia, arthralgias, and generalized painless lymphadenopathy.
  • Renal, hepatic, and ophthalmologic manifestations may be present.
  • Meningitis occurs in 30% of patients with secondary syphilis but may be asymptomatic. Symptomatic aseptic meningitis occurs in 1-2% of patients and is characterized by headache, stiff neck, nausea, and vomiting.
• Tertiary syphilis
  • Tertiary neurosyphilis presents with symptoms of meningitis or with focal deficits consistent with stroke. The mnemonic device "PARESIS" is an aid to recall the following types of symptoms:
    • Personality
    • Affect
    • Reflexes (eg, hyperactive)
    • Eye (eg, Argyll Robertson pupils)
    • Sensorium (eg, illusions, delusions, hallucinations)
    • Intellect (eg, decreased recent memory, orientation, judgment, insight)
    • Speech abnormalities
  • Syphilis at any stage can affect ears and eyes. Such involvement may be the only presentation, and, therefore, any patient with unexplained hearing loss, vestibular abnormalities, or ocular inflammation should be tested for treponemal antibodies. Syphilis should be considered in the differential diagnosis of inflammatory ocular hypertension syndrome. Patients may present with uveitis and serologic evidence of syphilis.
• Early onset congenital syphilis
  • Most affected infants are asymptomatic at birth and are identified only by routine prenatal screening. If untreated, symptoms develop within weeks or months. The typical stillborn or highly symptomatic newborn is born prematurely with an enlarged liver and spleen, skeletal involvement, and often pneumonia and bullous skin lesions.
  • The earliest signs of congenital syphilis may be poor feeding and snuffles (ie, syphilitic rhinitis).

Physical

• Primary syphilis
  • Primary syphilis is characterized by a painless papule at the site of inoculation that quickly erodes, leaving an ulcer (the chancre). The base of the ulcer is smooth, without exudate, and the borders are raised and firm.
  • Painless nonsuppurative enlargement of local lymph nodes accompanies the chancre and can persist for months.
  • Primary chancres heal within 3-6 weeks.
• Secondary syphilis
  • Mucocutaneous lesions are the most frequent signs of disease and strongly suggest the diagnosis. Discrete, macular, pink-to-red lesions are 3-10 mm in diameter and may spread to involve the entire body, including the palms, soles, and other locations.
  • Unlike the primary lesions, secondary lesions do not ulcerate. These lesions often evolve from macules into red papules and, in a few patients, finally progress to pustules. 
  • Vesicular lesions are conspicuously absent. 
  • Painless generalized lymphadenopathy is found in 85% of patients. 
  • Broad grayish plaques called condyloma lata may be found in warm, moist, intertriginous areas. 
  • Mucous patches are superficial, silver-gray erosions that occur on mucous membranes.
  • No pathognomonic signs are noted for ocular syphilis. Ocular involvement is usually evident beyond the primary stage of syphilis. Acute iridocyclitis in as many as 4% of patients with secondary syphilis.³
• Tertiary syphilis manifestations are divided into the following subgroups:
  • In benign tertiary syphilis, gummatous lesions are found in skin and bones but rarely in other organs. Gummas are considered benign because they rarely involve vital body structures.
  • Cardiovascular tertiary syphilis can cause aortitis, aortic aneurysm, coronary stenosis, aortic insufficiency, and myocarditis. Prevention of late syphilis can be adequately achieved by treating early syphilis. The treatments of choice are penicillin administration and surgical intervention in severe cases.
  • Meningitic tertiary neurosyphilis manifests with signs of meningitis and is differentiated from other causes of aseptic meningitis by a reactive result on a Venereal Disease Research Laboratory (VDRL) test of cerebrospinal fluid (CSF); this test is termed a CSF VDRL.
  • Late neurosyphilis may present with focal neurological findings suggestive of a stroke.
• Syphilis in pregnancy
  • Syphilis in pregnancy can lead to spontaneous abortion, stillbirth, premature delivery, or perinatal death. It can also cause significant morbidity during infancy, childhood, and adolescence. A very strict follow-up of pregnant women before delivery and an active approach to identify and treat exposed neonates born to infected mothers are strongly recommended.
  • A study in Nigeria has demonstrated the usefulness of syphilis screening during pregnancy and recommended that syphilis screening should be continued as part of routine antenatal testing.⁴
• Early onset congenital syphilis
  • Early manifestations of congenital infection vary and involve multiple organ systems. The most striking lesions affect the mucocutaneous tissues and bones. Mucous patches, rhinitis, and condylomatous lesions are highly characteristic features of mucous membrane involvement in congenital syphilis.
  • Nasal fluid is highly infectious. Snuffles are followed quickly by a diffuse maculopapular desquamative rash that involves extensive sloughing of the epithelium, particularly on the palms and soles and around the mouth and anus. In contrast to acquired syphilis, a vesicular rash and bullae may develop. These lesions are highly infectious.
  • Hepatomegaly is reported in almost 100% of cases, and biochemical evidence of liver dysfunction is usually observed.
• Late-onset congenital syphilis
  • Scarring from the early systemic disease causes late manifestations of congenital syphilis.
  • Manifestations include neurosyphilis and involvement of the teeth, bones, eyes, and the eighth cranial nerve.

Causes

• Syphilis transmission usually occurs transplacentally or by sexual contact.
• Vertical transmission of early syphilis during pregnancy results in a congenital infection in at least 50-80% of exposed neonates.
• Other modes of transmission include contact with contaminated blood or infected tissues.
• Children encounter 2 forms of syphilis: acquired syphilis, which is almost exclusively transmitted by sexual contact, and congenital syphilis, which results from transplacental transmission of spirochetes.
• A report of a syphilis outbreak in North Carolina described an association between crack cocaine and sex for drugs as a causative factor.⁵

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