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$Unique_ID{BRK04248}
$Pretitle{}
$Title{Syphilis, Congenital}
$Subject{Syphilis Congenital Lues Bejel Epidermolysis Bullosa Ectodermal
Dysplasias Jaundice Pinta Yaws}
$Volume{}
$Log{}

Copyright (C) 1991 National Organization for Rare Disorders, Inc.

841:
Syphilis, Congenital

** IMPORTANT **
It is possible that the main title of the article (Congenital Syphilis is
not the name you expected.  Please check the SYNONYMS listing to find
alternate names, disorder subdivisions, and related disorders covered by this
article.

Synonyms

     Lues

Information on the following diseases can be found in the Related
Disorders section of this report:

     Bejel
     Epidermolysis Bullosa
     Ectodermal Dysplasias
     Jaundice
     Pinta
     Yaws

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.

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 show up until several weeks or
months after birth and in some cases they 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 that 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 peg-shaped 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 Population

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.  This rise has been attributed to the use of "crack" cocaine and the
increase in prostitution to support drug abuse.  In New York City alone, the
number of congenital syphilis cases rose from 57 cases in 1986 to 1,000 cases
in 1989.

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.)

Therapies:  Standard

Congenital syphilis is preventable.  It occurs in infants who's 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.

Serologic tests may be seronegative 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.

Therapies:  Investigational

This disease entry is based upon medical information available through
February 1991.  Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate.  Please check with the agencies listed in the Resources section for
the most current information about this disorder.

Resources

For more information on Congenital Syphilis, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     Centers for Disease Control (CDC)
     1600 Clifton Road, NE
     Atlanta, GA  30333
     [phone]

     NIH/National Institute of Allergy and Infectious Diseases (NIAID)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

For local treatment centers contact any state or local health department
listed in your area phone directory.  These agencies can refer you to testing
facilities for venereal diseases.

References

INTERNAL MEDICINE, 2nd Ed.:  Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987.  Pp. 1719.

CECIL TEXTBOOK OF MEDICINE, 18th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1988.  Pp. 1718-19.

MEDICAL ASPECTS OF DEVELOPMENTAL DISABILITIES IN CHILDREN BIRTH TO THREE;
James A. Blackman, M.D., Editor; The University of Iowa, 1983.  Pp. 72.

Clinical Dermatology, 2nd Ed.:  Thomas P. Habif, M.D. Ed; The Mosby
Company., 1990.  Pp. 228-9.

THE EFFECTS OF SYPHILIS ON ENDOCRINE FUNCTION OF THE FETOPLACENTAL UNIT:
C.R. Parker Jr., et al.; Am J Obstet. Gynecol; (Dec. 1988, issue 159 (6)).
Pp. 1327-31.

CONGENITAL SYPHILI PRESENTING IN INFANTS AFTER THE NEWBORN PERIOD:  D.H.
Dorfman, and J.H. Glaser; N Engl Med; (Nov. 8, 1990, issue 323 (19)).  Pp.
1299-302.

CONGENITAL SYPHILI IN THE NEWBORN:  V. Chawla, P.B. Pandit, and F.K.
Nkrumah; Arch Dis Child; (Nov. 1988, issue 63 (11)).  Pp. 1393-4.

UMBILICAL CHORD SCHLEROSI AS AN INDICATOR OF CONGENITAL SYPHILIS:  S.
Knowles, and T. Frost; J Clin Pathol; (Nov. 1989)).  Pp. 1157-9.