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emedicine.medscape.com

Dermatologic Manifestations of
Rubella
Updated: Aug 10, 2017
Author: Peter C Lombardo, MD; Chief Editor: Dirk M Elston, MD

Overview

Background
Rubella is usually a mild viral illness involving the skin, the lymph nodes, and, less commonly, the joints. The most important
complication of rubella is congenital rubella syndrome (CRS).

Pathophysiology
Rubella is an RNA virus classified as a Rubivirus in the Togaviridae family.

Epidemiology
Frequency

Before the live rubella vaccine, epidemics of the disease were seen in young children (most common), adolescents, and young
adults every 5-9 years in winter and early spring. Since the rubella vaccine, the number of rubella cases has decreased
significantly.

Because of the morbidity of measles and rubella, the World Health Organization (WHO) maintains a worldwide Measles and
Rubella Laboratory Network (LabNet) to monitor the behavior of the viruses.[1] Because of the unreliability of clinical diagnosis,
laboratory surveillance is important in setting rubella elimination goals. Serum-based diagnostics remain the criterion standard;
however, programs are being developed using dried blood samples and oral fluid for confirmation of infection in those areas
where patients might resist venipuncture or where transportation and refrigeration of blood samples is difficult.

One major focus of infection in the recent past was unvaccinated adults.[2] Of concern was the high incidence of rubella in
unvaccinated Hispanic immigrants and congenital rubella syndrome in their offspring.[3] Individuals from Columbia, the
Dominican Republic, and Central America, where vaccination programs were just starting, were often susceptible to rubella. In
one study, 44% of congenital rubella syndrome cases were in Hispanic infants.[4] This was a public health concern. In an
outbreak in 1997-98 in New York State, the infections spread from the Hispanic community, along train and work lines, to involve
14 towns and 95 individuals.

Now fortunately, the Pan American Health Organization of the WHO announced that the endemic transmission of rubella has
ended in the Americas and that the Americas are free of endemic rubella.[5] The last confirmed case was in Argentina in 2009.
This end of endemic rubella was achieved by the high percentage of vaccination in the population. This does not mean that the
threat of rubella is over, as the remainder of the world is not rubella–free, and, with the mobility of the world population, cases of
rubella may still enter the United States.
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The Centers for Disease Control and Prevention (CDC)[6] states that a nonimmune woman with a rubella infection in the first
trimester stands a 90% chance of miscarriage, stillbirth, or transmittal of congenital rubella syndrome (CRS) to her baby. Five of
10 healthy babies do not receive the vaccine, even though it now costs about a dollar. The CDC maintains that 79 countries now
are using the vaccine more, but the WHO[7] maintains that 45 member states have not yet introduced the vaccine and two
regions (African and Eastern Mediterranean) have not yet set control targets. Therefore, it is possible for rubella to enter the
United States, and so it is essential to maintain a high level of immunization in the population to ensure herd immunity. Constant
vigilance remains crucial.

Race

Rubella has no racial predilection.

Sex

Both sexes are equally affected by rubella.

Age

Rubella primarily affects young children, but adolescents and young adults are also affected.

Prognosis
The prognosis is usually excellent, with the exception of congenital rubella syndrome (CRS).

Patient Education
Now that endemic rubella is eliminated in the Americas, the threat of rubella epidemics and subsequent congenital rubella
syndrome would be from cases of rubella that enter the United States from other areas of the world and infect the unvaccinated
clusters in this country. Therefore, the Measles and Rubella Initiative calls for monitoring the disease using effective
surveillance, developing and maintaining outbreak preparedness, communicating and engaging to build public confidence and
demand for immunization, and continuing research to improve cost-effective vaccinations and diagnostic tools.[8]

For patient education materials, see the articles Immunization Schedule, Adults and Immunization Schedule, Children.

Presentation

History
Studies on children at the New York Willowbrook State School in 1963, shortly after the isolation of the rubella virus, have shown
that the disease is spread by nasal droplet infection and has an incubation period of 14-19 days, with onset of a rash usually on
the 15th day.[9] The disease can be spread from a few days before to 5-7 days after the appearance of the exanthem. The virus
can be detected in the pharynx from 7 days before until 7 days after the rash. A viremia was detected from 7 days before until
the day of the rash, and the virus was present in the stool from 4 days before until 4 days after the rash. Isolating the virus from
children with subclinical infections was also possible.

Patients are most contagious when the rash is erupting. Rarely, the virus may be shed from the pharynx up to 15 days after the
appearance of the rash, in rapidly diminishing amounts, and it is very difficult to detect by culture after 5-7 days. Patients are not
considered clinically contagious after 7 days.

Infection usually confers lifelong immunity, but reinfection is occasionally detected serologically after the natural disease or a
vaccination upon reexposure to the virus and rarely results in clinical disease.
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Physical Examination
In children, a prodrome may not be present. The rash may be the first manifestation. In adults, fever, sore throat, and rhinitis
may be present. The exanthem begins as discrete macules on the face that spread to the neck, the trunk, and the extremities.
The macules may coalesce on the trunk. Appearance of the rash corresponds with the appearance of the rubella-specific
antibody. The exanthem lasts 1-3 days, first leaving the face, and may be followed by desquamation. On occasion, a nonspecific
enanthem (Forchheimer spots) of pinpoint red macules and petechiae can be seen over the soft palate and the uvula just before
or with the exanthem. Note the images below.

Young adult with macular rash.

Child with generalized eruption.

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The hallmark of rubella is the generalized, tender lymphadenopathy that involves all nodes, but which is most striking in the
suboccipital, postauricular, and anterior and posterior cervical nodes. It is most prevalent at the time of appearance of the
exanthem but may precede it by a week. The tenderness that accompanies this lymphadenopathy subsides rapidly; however,
the enlargement may last days or weeks.

Although less common in children, in adults, polyarthralgia and even polyarthritis may occur and rarely may persist longer than 2
weeks. It may resemble rheumatic fever or rheumatoid arthritis, with small and large joints being involved bilaterally with or
without swelling. The swelling can be very marked. Fifty percent of women may have arthralgias, and 10% have arthritis, 3 days
post rash with the natural infection or within 2-6 weeks after a vaccination.

Rarely, recurrent episodes of inflammation of the fingers, the wrists, and the knees can continue for more than a year. Very
rarely, a syndrome of low-grade fever, chronic fatigue, and myalgias can persist for months or years. The pathogenesis of the
arthritis is not known. The virus can be isolated from joint effusions in acute and recurrent cases. Peripheral blood mononuclear
cells may harbor the rubella virus in chronic arthritis. Test results for rheumatoid arthritis are negative.

Causes
Rubella is an RNA virus classified as a Rubivirus in the Togaviridae family.

Complications
Complications are rare with rubella in healthy infants and adults. Rarely, encephalitis or peripheral neuritis may occur; however,
recovery is usually complete without sequelae. Thrombocytopenia usually resolves within a month, but it may result in purpura,
epistaxis, and intestinal bleeding.

Congenital rubella syndrome (CRS)

This the most severe and important complication of rubella and occurs in the fetus of a pregnant woman without immunity to the
virus. Of infants infected in the first trimester, 50% are affected, and the severity depends on how early the infection occurs.
Note the following:

The most common abnormalities are ophthalmologic in nature (eg, cataracts, retinopathy).

Cardiac abnormalities (eg, patent ductus arteriosus, pulmonary stenosis) may be seen.

Auditory involvement may be present as sensorineural deafness.

Neurologic disorders (eg, meningoencephalitis, mental retardation with behavioral disorders) may occur.

If infection occurs after organ development, a variable picture may be seen, with hepatitis, splenomegaly, pneumonitis,
myocarditis, and/or osteomyelitis.

If the bone marrow is affected, thrombocytopenia with purpura and petechiae occur. Bizarre purple macules and papules, which
represent persistent dermal (extramedullary) hematopoiesis, are seen in the skin. This appearance is known as blueberry muffin
baby. Note the image below.

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Blueberry muffin newborn with lesions on the forehead.

An infant who is affected may continue to shed the virus for up to 1 year. At least 85% of infants who are affected shed the virus
at 1 month, and 1-3% do so at 1 year. Therefore, these individuals should be considered contagious for at least 1 year and
should be considered an exposure threat to nonimmune pregnant women, unless nasopharyngeal or urine culture results are
repeatedly negative.

Pregnancy

No adequate treatment is available for pregnant women exposed to rubella. Immunoglobulin is not recommended unless
termination of the pregnancy is not an option because cases of congenital rubella syndrome have occurred in infants born to
mothers who received immunoglobulin shortly after exposure.

DDx

Differential Diagnoses
Dermatologic Manifestations of Kawasaki Disease

Drug Eruptions

Erythema Infectiosum

Measles

Roseola Infantum

Scarlet Fever

Workup

Workup

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Laboratory Studies
In a healthy child or adolescent, the diagnosis of rubella is made on a clinical basis, and a laboratory workup is not necessary.

A WBC count, if performed, may be lower than normal, as in many viral infections, with increased percentages in the
lymphocyte count. In those very rare cases in which encephalitis is present, lymphocytes are present in the cerebrospinal fluid
(CSF). If necessary, rubella virus can be cultured from the nasopharynx, blood, urine, and CSF.[10]

If the diagnosis is in doubt, a rising titer of immunoglobulin M (IgM) antibody over a 2-week period indicates a recent infection.
Two specific antibodies are associated with Rubella. The first to appear is IgM antibody, which rises and peaks 7-10 after
infection and then tapers off after several weeks. One exception to this rule is in the infected newborn, in whom IgM may be
detected for months to a year. The immunoglobulin G (IgG) antibody develops more slowly but remains positive for life. It
confers immunity against repeat infection. Therefore, the presence of IgM antibody indicates a recent infection, whereas IgG
antibody indicates an old infection and immunity. IgG antibody may also indicate immunity caused by the measles, mumps,
rubella (MMR) vaccine.

From a public health standpoint, attempting to confirm a rubella infection in a pregnant woman or a newborn infant is important;
a pregnant woman exposed to rubella should be tested immediately for a rubella-specific antibody. The presence of rubella-
specific IgG is evidence that the patient is immune. If the test result is negative, repeat the test again in 3-4 weeks, and repeat
the test on the first specimen. If an antibody is present in the second test and not in the first test, an infection has occurred. If the
second test result is negative, repeat the test in 6 weeks, and, again, test it with the first specimen. A negative test result in both
specimens means an infection has not occurred, whereas if the last specimen is positive and the first specimen is negative, then
an infection has occurred.

An infant with congenital rubella syndrome shows the IgG antibody from the mother, which disappears in a few months, and an
elevated IgM antibody level because of antibody production by the infant. The presence of the IgM antibody usually indicates
recent infection because IgM does not cross the placenta from the mother as does IgG. After 1 year, confirming the diagnosis of
congenital rubella syndrome in an infant with serology alone is very difficult.

Histologic Findings
The histopathologic features of the skin are those of the Togaviruses, namely, a light perivascular infiltrate of lymphocytes with
mild endothelial swelling. If petechiae or purpura are present clinically, extravasation of erythrocytes may be observed.

Treatment

Medical Care
No specific treatment is available for rubella. The disease is usually self-limited. Rest and oral fluids are appropriate. Individuals
may remain contagious for 7 days after the onset of the rash, and they should be appropriately isolated from work, school, or
other public settings.

Prevention
While rubella usually has a mild clinical course, the sequel of congenital rubella syndrome can be devastating. The main
defense against congenital rubella syndrome is a comprehensive vaccination plan nationally and internationally.

In 2003, an increase occurred in cases of rubella in England and Wales that was the direct result of a decrease in the
vaccination rate, because of perceived adverse effects of the MMR vaccination, namely autism.[11] A population-based study in
Denmark involving more than a half million children showed no significant increase in autism in children who had received the
MMR vaccination compared with those who were not vaccinated.[12] These results have been confirmed by other studies.[13]
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The idea that the MMR vaccine causes autism is a misconception that must be overcome. Because of the lack of valid
evidence, the Federal "vaccine court" now rejects claims that the MMR vaccine causes autism.[14] Parents must be cautioned
that by not vaccinating their children because of this misconception, they may be exposing them and others to the very real
complication of congenital rubella syndrome. Physicians, who are usually the primary source of correct information for their
patients, must work with these parents to understand and attempt to minimize these fears.[15]

The Measles and Rubella Initiative Strategic Plan 2010-2020,[8] which is indorsed by the WHO, the CDC and other global
health organizations, models its plan to eliminate these viral diseases globally based on the experience in the Americas, where
endemic rubella is now eliminated. The mainstay of this strategy is to achieve and maintain high levels of population immunity
by providing high vaccination coverage with two doses of measles- and rubella-containing vaccines. One obstacle to achieving
this goal is the increase in vaccine refusals for "personal belief" in the United States, resulting in geographic clusters of refusals
and the possibility of outbreaks. This increases the risk of infection for those who refuse vaccination and for those who are not
candidates for it because of age, immunosuppression, or some other contraindication.[15] Fortunately, some states are now
passing legislation that removes personal belief as a reason not to be vaccinated.

The live rubella vaccine is an RA27/3 strain grown in human diploid cell cultures. The live rubella vaccine is usually given with
the measles and mumps vaccine (ie, MMR vaccine) at age 12-15 months and again at school entry at age 4-6 years. The MMR
vaccine can now be combined with the varicella vaccine (ie, MMRV vaccine) as one injection for ease of administration.[16]

The Centers for Disease Control and Prevention (CDC) have issued the following recommendations[17] :

One or more doses of MMR vaccine should be given to adults who were born during or after 1957 unless they have a
medical contraindication, documentation of one or more doses, history of measles diagnosed by a healthcare provider, or
laboratory evidence of immunity.

A second dose of MMR is recommended for adults recently exposed in an outbreak setting, previously vaccinated with a
killed measles vaccine, or vaccinated with an unknown type of measles vaccine during 1963-1967.

Second doses should also be given to students in postsecondary educational institutions, who work in a healthcare
facility, or who plan international travel.

Women of childbearing age, regardless of birth year, should be assessed for rubella immunity and counseled concerning
congenital rubella syndrome. Women without immunity should receive the MMR vaccine upon completion or termination
of pregnancy and before discharge from the healthcare facility.

A few contraindications to vaccination should be noted, as follows:

Pregnant women should not be vaccinated; however, vaccination of her household contacts is not contraindicated
because no evidence indicates that the virus would spread to her even though the vaccinee may shed the virus for 1-4
weeks.

Patients receiving hyperimmune globulin should avoid vaccination 2 weeks before and 3 months after its administration.
If hyperimmune globulin is given, the patient should be tested for immunity at least 8 weeks following vaccination.

Patients who are seriously ill should not be vaccinated; however, fever in itself is not a contraindication.

Patients with severe altered immunity should not be vaccinated. Patients on immunosuppressive therapy should not be
vaccinated for 3 months. Patients with HIV disease may be vaccinated if they are not severely immunocompromised.

Also see the Recommended Immunization Schedule for Adults Aged 19 Years or Older, United States, 2017 from the CDC.[18]

Adverse reactions to vaccination include rash, fever, and/or lymphadenopathy developing 5-12 days later in 5-15% of children.
Joint pain is rare but is more prevalent in women and is usually less severe than that seen in the naturally occurring disease.
Rarely, transient peripheral neuritis symptoms have been reported.

The combined MMRV vaccine (ProQuad) has been shown to be associated with an increased risk of febrile seizures occurring
5-12 days following vaccination, at a rate of 1 in 2300-2600 in children aged 12-23 months compared with a separate MMR
vaccine and a varicella vaccine administered simultaneously.[19, 20] As a result, the CDC Advisory Committee on Immunization
Practices (ACIP) recommends that separate MMR and varicella vaccines be used for the first dose, although providers or
parents may opt to use the combined MMRV for the first dose after counseling regarding this risk.[21] MMRV is preferred for the
second dose (at any age) or the first dose if given at age 48 months or older.

Data from postlicensure studies do not suggest that children aged 4-6 years who received the second dose of MMRV vaccine
had an increased risk for febrile seizures after vaccination compared with children of the same age who received the MMR
vaccine and varicella vaccine administered as separate injections at the same visit.[21]

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Medication

Medication Summary
No specific medication is available for rubella, except that given for symptomatic relief. High fever and polyarthralgia are rare in
children; however, if present, acetaminophen may be used. In adults, in whom fever and polyarthralgia are more common,
acetaminophen, aspirin, or other nonsteroidal anti-inflammatory drugs (NSAIDs) are useful.

Analgesics, salicylate

Class Summary
These agents can reduce inflammation and fever.

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

Aspirin treats mild to moderate pain and headache. It inhibits prostaglandin synthesis, which prevents the formation of platelet-
aggregating thromboxane A2. Aspirin acts on the heat-regulating center of the hypothalamus and vasodilates peripheral vessels
to reduce fever.

Nonsteroidal anti-inflammatory agents

Class Summary
These agents have analgesic and antipyretic activities. Their mechanism of action is not known, but they may inhibit cyclo-
oxygenase activity and prostaglandin synthesis. Other mechanisms (eg, inhibition of leukotriene synthesis, lysosomal enzyme
release, lipoxygenase activity, neutrophil aggregation, various cell membrane functions) may also exist. Although its effects in
the treatment of pain tend to be patient-specific, ibuprofen is usually DOC for initial therapy.

Ibuprofen (Ibuprin, Advil, Motrin)

Ibuprofen is the drug of choice for mild to moderate pain. It inhibits inflammatory reactions and pain by decreasing prostaglandin
synthesis. It is one of the few NSAIDs indicated for reduction of fever.

Contributor Information and Disclosures

Author

Peter C Lombardo, MD Associate Clinical Professor, Department of Dermatology, Columbia University College of Physicians
and Surgeons; Private Practice, Sutton Place Dermatology, PC

Peter C Lombardo, MD is a member of the following medical societies: American Academy of Dermatology, American Medical
Association, Dermatologic Society of Greater New York, New York Academy of Medicine, New York State Society of
Dermatology and Dermatological Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

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Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center,
Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical
Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at
San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of
South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at
Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical
Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous Chief Editor, William D.
James, MD, to the development and writing of this article.

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