A contagious systemic disease caused by the slender, spiral organism, Treponema pallidum (T. pallidum). The disease encompasses sequential clinical stages and years of latency.

The T. pallidum is a delicate, slender, spiral organism. It is identified by a darkfield microscope or fluorescent techniques. The organism cannot survive for long periods outside the human body.

In acquired syphilis, T. pallidum enters through the mucous membranes or skin, then moves to the regional lymph nodes, and rapidly invades the body. In all stages of syphilis, swelling and excess of the endothelium of smaller blood vessels develop, and the vessels become narrow and obliterated (endarteritis obliterans). In late syphilis, T. pallidum elicits a reaction causing masses, ulcerations, and necrosis. Inflammation may subside despite progressive damage.

Signs and symptoms of the primary, secondary, latent, or late (tertiary) stages of syphilis are as follows:

• Primary Stage

  • The primary lesion heals within 4 to 8 weeks in untreated patients. The regional lymph nodes usually enlarge painlessly, and are nontender and firm. Chancres appear on the penis, anus, and rectum in men, and on the vulva, cervix, and perineum in women. Chancres may also occur on the lips, but rarely occur on the hands or other parts of the body.

• Secondary Stage

  • Skin rashes usually appear within 6 to 12 weeks after infection, and are more obvious after 3 to 4 months. A quarter of the patients have a residual primary chancre. The lesions may be transient or may occur for months at a time. If untreated, they may heal, but new chancres form within weeks or months. Often, generalized, nontender, firm, discrete and enlarged lymph nodes are found in the liver and spleen. Over 80% of patients have mucous membrane lesions, 50% have generalized lymphadenopathy, and about 10% have lesions of the eyes, bones, joints, meninges, kidneys, liver, and spleen.

  • Mild symptoms of fever, malaise, headache, anorexia, nausea, achy bones, and fatigability are often present. Anemia, jaundice, and albumin in the urine are common. Acute syphilitic meningitis may develop which is manifested by headache, neck stiffness, cranial nerve lesions, deafness, and occasionally, swelling of the optic disc.

  • Syphilitic skin rashes may simulate various skin conditions. Usually, they are symmetrical and appear more on the flexor surfaces of the body, the palms, and the soles. The rashes generally occur in crops as patches, pimples, pus-filled, or scaly lesions.

• Latent Stage

  • This stage may resolve spontaneously in a few years or last for the rest of the patient's life. Less than 2 years after infection, infectious mucous membrane relapses may occur. After 2 years, contagious lesions rarely develop, and the patient appears normal. One-third of untreated persons will develop late syphilis; perhaps not until many years after the initial infection. Patients who may have received antibiotics for other conditions may be cured, which could account for the rarity of late-stage disease in developed countries.

• Late or Tertiary Stage

  • The lesions that occur in this phase are:

  • 1. Benign tertiary syphilis of the skin, bone, and viscera

    2. Cardiovascular syphilis

    3. Neurosyphilis

The erythrocyte sedimentation rate (ESR) and C-reactive protein tests are done. The white blood cell (WBC) count reaches 12,000 to 20,000/?L and the serum C-reactive protein is abnormally high.

All stages of syphilis are treated with specific antibiotics. Cephalosporins have also been used in treatment. Various preparations are used to treat the different forms of syphilis. Treatment of syphilis of the eyes (ocular), or neurosyphilis in HIV-infected persons may be more difficult.

The typical lesion of benign tertiary syphilis is a gumma. This inflammatory mass progresses to necrosis and fibrosis. It usually remains local, but may extend into an organ or tissue. Gummas are slow in progression, heal gradually, and leave scars. They may develop in the skin, causing nodules, ulcers, or scaly eruptions.

Gummas occur in submucosal tissues and can possibly lead to perforation of the palate or septum. They are most common on the leg, upper trunk, face, and scalp. However, they may occur almost anywhere. Benign tertiary syphilis of the bones results in either infection in the bones, or osteitis with destructive lesions which are painful especially at night.

In cardiovascular syphilis, a spindle-shaped aneurysm of the ascending or transverse aorta may develop. The coronary opening may narrow, or aortic valvular insufficiency may appear 10 to 25 years after the initial infection.

Neurosyphilis without symptoms occurs prior to symptomatic neurosyphilis. It occurs in about 15% of those originally diagnosed with latent syphilis, in 12% of those with cardiovascular syphilis, and in 5% of those with benign tertiary syphilis.

Meningovascular neurosyphilis involves the brain. Headache, dizziness, poor concentration, exhaustion, insomnia, neck stiffness, and blurred vision signal the condition. Mental confusion; epileptic-like attacks; edema and inflammation of the optic nerve; aphasia; (inability to communicate); and paralysis of a single limb or side of the body are also manifestations.

Cranial nerve paralysis and pupillary abnormalities usually indicate basilar meningitis. The Argyll Robertson pupil occurs almost exclusively in neurosyphilis. The symptom presents as a small, irregular pupil that contracts normally during accommodation, but has no reaction to light.

Neurosyphilis, involving essential parts of the neurological system, generally affects patients in their 40s or 50s. It is manifested by progressive deterioration in behavior, and may mirror a psychiatric illness or Alzheimer's disease. Convulsions, difficulty communicating (verbally or otherwise), or transient weakness on one side of the body may occur. Irritability, difficulty in concentrating, deterioration of memory, defective judgment, headache, insomnia, or fatigue and lethargy more commonly appear. The patient's hygiene and grooming deteriorate. Emotional instability resulting in lack of strength, depression, and delusions of grandeur with lack of insight, may occur.

Physical alterations that may occur include: tremors of the mouth, tongue, outstretched hands, and whole body; pupillary abnormalities; difficulty speaking; brisk tendon reflexes; and in some cases, extensor plantar responses. Handwriting is usually shaky and illegible. Stabbing pain in the back and legs is the first and most characteristic symptom that recurs on an irregular basis. Defective muscle coordination, hyperesthesia, and paresthesia may also occur. Bladder sensation is lost which leads to urine retention, incontinence, and recurrent infections. Impotence is also a common occurrence.

Pains in various organs arise from lesions located in the particular area. Gastric crises with vomiting are most common. Rectal, bladder, and laryngeal crises also occur. Trophic lesions of the skin or surrounding joint tissue may develop in the later stages of the disease. The soles of the feet may develop trophic ulcers. A condition that results in painless joint degeneration, bony swelling and abnormal range of movement known as Charcot's arthropathy, is also common.

This disease shall be granted service connection although not otherwise established as incurred in or aggravated by service if manifested to a compensable degree within the applicable time limits under §3.307 following service in a period of war or following peacetime service on or after January 1, 1947, provided the rebuttable presumption provisions of §3.307 are also satisfied [38 CFR Book B §3.309 [Disease subject to presumptive service connection], §3.309(a) [chronic disease].

Rate under the appropriate body system any residual disability of infection, which includes, but is not limited to, diseases of the nervous system, vascular system, eyes, or ears (see DC 7004, DC 8013, DC 8014, DC 8015, and DC 9301) (38 CFR 4.188b [Schedule of ratings-infectious diseases, immune disorders and nutritional deficiencies]).