Pediatric Syphilis Clinical Presentation

Most recognized syphilitic disease in children is congenital. Medical professionals should assume that children with acquired syphilis have been infected through sexual abuse, unless another mechanism of transmission is identified.

Syphilis-especially in its later stages-can have numerous and complex manifestations and may resemble a number of other diseases. Indeed, William Osler called syphilis "the Great Imitator."

Primary syphilis

The primary lesion, called a chancre, is painless. It usually develops at the site of the inoculation, at 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. If the chancre occurs on the vulva or in the mouth, it is often overlooked by the patient and partner.

Secondary syphilis

Because of the widespread dissemination of spirochetes, frequent constitutional symptoms include the following:

• Fever

• Malaise

• Pharyngitis

• Rash

• Anorexia

• Arthralgias

• Generalized painless lymphadenopathy

Renal, hepatic, and ophthalmologic manifestations may be present.

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 symptoms and signs:

• P ersonality

• A ffect

• R eflexes (eg, hyperactive)

• E ye (eg, Argyll Robertson pupils)

• S ensorium (eg, illusions, delusions, hallucinations)

• I ntellect (eg, decreased recent memory, orientation, judgment, insight)

• S peech 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.

Go to Interstitial Keratitis for more complete information on this topic.

Syphilis in HIV-exposed infants

Several studies have demonstrated atypical features of congenial syphilis in infants simultaneously infected with HIV and treponema. These features include fever, cough, blister or nonblister rash, and sores around the corners of the mouth. One should have a high index of suspicion with any such findings in a newborn that cannot be explained by any one entity.

Primary syphilis

Primary syphilis is characterized by a nontender 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 spontaneously heal within 3-6 weeks.

Secondary syphilis

Mucocutaneous lesions are the most frequent signs of secondary syphilis 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. (See the photographs below.)

Abnormal cerebrospinal fluid (CSF) examination results occur in 30% of patients with secondary syphilis, but the patient may be asymptomatic.

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. ^[6]

Tertiary syphilis

Tertiary syphilis manifestations are divided into the following subgroups: benign, cardiovascular, and late.

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. (See the photographs below.)

Cardiovascular tertiary syphilis can cause aortitis, aortic aneurysm, coronary stenosis, aortic insufficiency, and myocarditis.

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. ^[7] Any woman who delivers a stillborn infant after 20 weeks' gestation should be tested for syphilis. Infected infants may experience significant morbidity during infancy, childhood, and adolescence.

Routine prenatal screening for syphilis remains the most important factor in identifying infants at risk of developing congenital syphilis. Screening is legally required at the beginning of prenatal care in all states in the United States.

Screen women at high risk for syphilis more frequently because they may have repeat infections during pregnancy or may become reinfected late in pregnancy. A study in Nigeria has demonstrated the usefulness of syphilis screening during pregnancy. These researchers recommended that syphilis screening should be continued as part of routine antenatal testing. ^[8]

Early-onset congenital syphilis (diagnosed before or at age 2 y)

Early manifestations of congenital infection vary and involve multiple organ systems. About 60% of infants born with congenital syphilis are asymptomatic at birth. Symptoms develop within the first 2 months of life. In symptomatic infants, the most common physical finding, reported in almost 100% of cases, is hepatomegaly; biochemical evidence of liver dysfunction is usually observed.

The other common findings are skeletal abnormalities, rash, and generalized lymphadenopathy. Radiographic abnormalities, periostitis or osteitis, involve multiple bones and are seen in the vast majority of symptomatic infants, but they also can be found in on fifth of infants with no symptoms or relevant findings on physical examination. Sometimes, the lesion is painful and an infant will favor an extremity (pseudopalsy). The rash is maculopapular and may involve palms and soles. In contrast to acquired syphilis, a vesicular rash and bullae may develop. These lesions are also highly contagious.

Mucosal involvement may present as rhinitis ("snuffles"). Nasal secretions are highly contagious.

Hematological abnormalities include anemia and thrombocytopenia. Some have leukocytosis. Abnormal CSF examination is seen in a half of symptomatic infants but also can be found in 10% of those who are asymptomatic.

Late-onset congenital syphilis (diagnosed >2 y)

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, as follows:

• Bone involvement - Saber shins, saddle nose

• Teeth involvement - Notched, peg-shaped incisors (Hutchinson teeth)

• Pigmentary involvement - Linear scars (rhagades) at the corners of the mouth

• Interstitial keratitis - Presents in the first or second decade of life

• Sensory-neural hearing loss (eighth cranial nerve deafness) - Presents between age 10 and 40 years.

• Classic Hutchinson triad - (1) defective incisors, (2) interstitial keratitis, (3) eighth cranial nerve deafness

Go to Interstitial Keratitis for more complete information on this topic.