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Syphilis, Congenital

Synonyms of Syphilis, Congenital

  • Lues, Congenital

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Congenital syphilis is a chronic infectious disease caused by a spirochete (treponema pallidum) acquired by the fetus in the uterus before birth. Symptoms of this disease may not become apparent until several weeks or months after birth and, in some cases, may take years to appear. Congenital syphilis is passed on to the child from the mother who acquired the disease prior to or during pregnancy. The infant is more likely to have congenital syphilis when the mother has been infected during pregnancy although it is not uncommon for an infant to acquire congenital syphilis from a mother who was infected prior to pregnancy. Symptoms of early congenital syphilis include fever, skin problems and low birth weight. In late congenital syphilis, the symptoms of the disease do not usually become apparent until two to five years of age. In rare cases, the disease may remain latent for years with symptoms not being diagnosed until well into adulthood.

Symptoms

Congenital syphilis is acquired by the fetus when the treponema pallidum spirochete is present in the mother. Pregnant women with syphilis may have a reduction in estrogen while serum progesterone levels may increase. Symptoms of early congenital syphilis usually appear at three to fourteen weeks of age but may appear as late as age five years. Symptoms may include inflammation and hardening of the umbilical chord, rash, fever, low birth weight, high levels of cholesterol at birth, aseptic meningitis, anemia, monocytosis (an increase in the number of monocytes in the circulating blood), enlarged liver and spleen, jaundice (yellowish color of the skin), shedding of skin affecting the palms and soles, convulsions, mental retardation, periostitis (inflammation around the bones causing tender limbs and joints), rhinitis with an infectious nasal discharge, hair loss, inflammation of the eye's iris and pneumonia.

Symptoms of late congenital syphilis usually present themselves after age five and may remain undiagnosed well into adulthood. The characteristics of late congenital syphilis may be bone pain, retinitis pigmentosa (a serious eye disease), Hutchinson's triad which is characterized by pegshaped upper central incisors (teeth), and interstitial keratitis which consists of blurred vision, abnormal tearing, eye pain and abnormal sensitivity to light, saddle nose, bony prominence of the forehead, high arched palate, short upper jawbone, nerve deafness and fissuring around the mouth and anus.

Causes

Congenital syphilis is a chronic infectious disease caused by the spirochete treponema pallidum and transmitted by an infected mother to the fetus in the womb. Adults transmit syphilis through sexual contact. (For information on syphilis in adults choose "Syphilis" as your search term in the Rare Disease Database).

Affected Populations

In 1998, the National Syphilis Elimination Plan went into effect. One of the goals of this plan was to reduce the incidence of congenital syphilis (CS) to fewer than 40 cases per 100,000 live births. The incidence of congenital syphilis in newborns under a year old rose in the United States from 180 cases in 1957 to 422 cases in 1972. More recently there has been a dramatic increase of congenital syphilis, especially in urban areas, that has been attributed in part to crack cocaine use. In the year 2000, the incidence rate was about 13.4 per 100,000 live births, which represented about 529 cases.

Related Disorders

Symptoms of the following disorders can be similar to those of Congenital Syphilis. Comparisons may be useful for a differential diagnosis:

Bejel, or endemic syphilis, is an infectious disease caused by an organism (treponema pallidum II) related to and identical in appearance to that causing venereal syphilis. This infection causes lesions of the skin and bone and is common among children in the Mediterranean countries of the Middle East, northern Africa, parts of eastern Europe, Arabia, subsaharan Africa, and Southeast Asia. In the United States, however, it is rare. Bejel is transmitted by physical, non-sexual contact and the sharing of eating and drinking utensils. (For more information on this disease choose "Bejel" as your search term in the Rare Disease Database)

Epidermolysis Bullosa is the name of a group of rare, hereditary skin diseases in which blisters (vesicles) develop usually following trauma. Severe forms of the disease may include involvement of the mucous membranes and may leave scars and contractures on healing. The shedding or absence of skin during infancy may be confused with the diagnosis of congenital syphilis. (For more information on Epidermolysis Bullosa choose "Epidermolysis" as your search term in the Rare Disease Database)

Ectodermal Dysplasias are a group of hereditary, non-progressive skin diseases. The skin, it's derivatives, and some other organs are involved. A predisposition to respiratory infections, due to a somewhat depressed immune system, and to defective mucous glands in parts of the respiratory tract, is the most life threatening characteristic of this group of disorders. Symptoms include eczema, poorly functioning sweat glands, sparse or absent hair follicles, abnormal hair, disfigured nails, and difficulties with the nasal passages and ear canals. Skin is satiny smooth, prone to rashes, and slow to heal. Commonly, the teeth fail to develop properly. Other complications may include hearing deficit, loss of sight, mental retardation, limb abnormalities, cleft palate and lip, and urinary tract abnormalities. Allergies are common, as are bronchitis and pneumonia. (For more information on Ectodermal Dysplasias choose "Ectodermal" as your search term in the Rare Disease Database.)

Jaundice is a yellow discoloration of the skin, tissues and certain body fluids caused by excess circulating bilirubin (reddish yellow pigment occuring in the urine, bile, blood and gallbladder). A wide range of liver disorders may cause jaundice. An evaluation based on physical examination, history, and routine laboratory tests will identify the cause of jaundice. Treatment of the underlying disorder is required. (For more information on diseases that cause Jaundice choose "Jaundice" as your search term in the Rare Disease Database.)

Pinta is an infectious disease caused by the microorganism treponema carateum and is closely related to the microorganism which causes some venereal disease. This disease is transmitted nonsexually and is characterized by rashes and discoloration of the skin. Small bumps develop and within several months reddish, scaly areas appear most often on the face, hands, and feet. It is common in the hot lowlands of Central and South America, but is rare in the United States. (For more information on this disease choose "Pinta" as your search term in the Rare Disease Database)

Yaws is a nonvenereal infectious disease caused by the microorganism treponema pertenue which is related to syphilis. This disorder is common in children and is characterized by skin and bone lesions. Yaws is rarely found in the United States but is common among children in the humid tropics of Africa, South and Central America, the West Indies, and the Far East. (For more information on this disease choose "Yaws" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
The diagnosis of CS may be delayed because the symptoms are not always evident. However, any child of a mother infected with syphilis should be suspected for congenital syphilis. If the signs and symptoms are not obviously those of CS, then further testing will be required, including (1) the examination of blood samples for antibodies to the spirochete infection and (2) identification of the syphilis bacterium under a light microscope.

Treatement
Congenital syphilis is preventable. It occurs in infants whose mothers have not been treated for the disease prior to or during pregnancy. When the infection is very recent, the disease may not show up in the infant. Therefore, it is important to have the infant tested again later on if the mother has been diagnosed with syphilis.

It is possible that blood tests (serologic tests) may be negative during pregnancy. Symptoms may then show up when the infant is 3-14 weeks of age. In these cases the mother probably acquired the infection during the later part of her pregnancy.

The most effective treatment for syphilis in the mother, as well as congenital syphilis in the infant, is penicillin. In some cases other antibiotics may be used. Interstitial keratitis may be treated with corticosteroid drugs and atropine drops. An ophthalmologist should be consulted.

If nerve deafness is present a combination of penicillin and corticosteroids may be prescribed.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Syphilis, Congenital Resources

Organizations:

References

TEXTBOOKS
Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:2191-93.

Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1447, 1509.

Jones III H, Wentz AC, Burnett LS, eds. Novak's Textbook of Gynecology. 11th ed. William & Wilkins, Baltimore, MD; 1988:577.

Mandell GL, Bennett JE, Dolan R, eds. Mandell, Douglas and Bennett's Principles and Practice of Infectious Diseases. 4th ed. Churchill Livingstone Inc. New York, NY; 1995:2119, 2126.

REVIEW ARTICLES
Peeling RW, Ye H. Diagnostic tools for preventing and managing maternal and congenital syphilis: an overview. Bull World Health Organ. 2004;82:439-46.

Berman SM. Maternal syphilis: pathophysiology and treatment. Bull World Health Organ. 2004;82:433-38.

Saloojee H, Velaphi S, Goga Y, et al. The prevention and management pof congenitalsyphilis: an overview and recommendations. Bull World Health Organ. 2004;82:410-16.

Schmid G. Economic and programmatic aspects of congenital syphilis prevention. Bull World Health Organ. 2004;82:402-09.

Dobson S. Congenital syphilis resurgent. Adv Exp Med Biol. 2004;549:35-40.

Carey JC. Congenital syphilis in the 21st century. Curr Womens Health Rep. 299-302

Hariprasad SM, Moon SJ, Allen RC, et al. Keratopathy from congenital syphilis. Cornea. 2002;21:608-09.

Walker DG, Walker GJ. Forgotten but not gone: the continuing scourge of congenital syphilis. Lancet Infect Dis. 2002;2:432-36.

FROM THE INTERNET
Congenital Syphilis --- United States, 2000. CDC, MMWR Weekly. July 13, 2001;573-77.
www.cdc.gov/mmrw/preview/mmwrhtml/mm5027a1.htm

Congenital Syphilis --- United States, 1998. CDC, MMWR Weekly. September 03, 1999;757-61.
www.cdc.gov/mmrw/preview/mmwrhtml/mm4834a3.htm

Syphilis. Health Matters. National Institute of Allergy and Infectious Diseases (NIAID). November 2002. 6pp.
www.niaid.nih.gov/factsheets/stdsyph.htm

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2009/05/15 00:00:00 GMT+0

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