7/30/2017 Pediatric Rubella Clinical Presentation: History, Physical, Postnatal Rubella

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Pediatric Rubella Clinical Presentation

Updated: Feb 09, 2017
Author: Elias Ezike, MD; Chief Editor: Russell W Steele, MD more...

PRESENTATION

History
Postnatal rubella
Rubella virus is transmitted from person to person via the aerosolized particles from the respiratory
tract. A history of exposure may not be present. Individuals may acquire the infection from a
completely asymptomatic patient or from an individual shedding the virus during the incubation period.

The incubation is usually 14-21 days after exposure to a person with rubella.

Prodromal symptoms are unusual in young children but are common in adolescents and adults.

The following signs and symptoms usually appear 1-5 days before the onset of rash:

Eye pain on lateral and upward eye movement (a particularly troublesome complaint)
Conjunctivitis
Sore throat
Headache
General body aches
Low-grade fever
Chills
Anorexia
Nausea
Tender lymphadenopathy (particularly posterior auricular and suboccipital lymph nodes)
Forchheimer sign (an enanthem observed in 20% of patients with rubella during the prodromal
period; can be present in some patients during the initial phase of the exanthem; consists of
pinpoint or larger petechiae that usually occur on the soft palate)

Congenital rubella

Congenital rubella history focuses on the following:

The number of weeks of pregnancy when maternal exposure to rubella occurred (The risk of
congenital rubella syndrome is higher if maternal exposure occurs during the first trimester.)
Maternal history of immunization or medical history of rubella
Evidence of intrauterine growth retardation during pregnancy
Manifestations suggestive of congenital rubella syndrome in a child

Physical
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Physical findings are based on the type of rubella.

Postnatal Rubella
Rash
The exanthem of rubella consists of a discrete rose-pink maculopapular rash ranging from 1-4 mm.
See the image below.

Image in a 4-year-old girl with a 4-day history of low-grade fever, symptoms of an upper respiratory tract infection,
and rash. Courtesy of Pamela L. Dyne, MD.
View Media Gallery

Rash in adults may be quite pruritic.

The synonym "3-day measles" derives from the typical course of rubella exanthem that starts initially
on the face and neck and spreads centrifugally to the trunk and extremities within 24 hours. It then
begins to fade on the face on the second day and disappears throughout the body by the end of the
third day.

Temperature

Fever is usually not higher than 38.5C (101.5F).

Lymph nodes
Enlarged posterior auricular and suboccipital lymph nodes are usually found on physical examination.

Mouth

The Forchheimer sign may still be present on the soft palate.

Congenital Rubella Syndrome

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The classic triad presentation of congenital rubella syndrome consists of the following:

Sensorineural hearing loss is the most common manifestation of congenital rubella syndrome. It
occurs in approximately 58% of patients. Studies have demonstrated that approximately 40% of
patients with congenital rubella syndrome may present with deafness as the only abnormality
without other manifestations. Hearing impairment may be bilateral or unilateral and may not be
apparent until the second year of life.
Ocular abnormalities including cataract, infantile glaucoma, and pigmentary retinopathy occur in
approximately 43% of children with congenital rubella syndrome. Both eyes are affected in 80%
of patients, and the most frequent findings are cataract and rubella retinopathy. Rubella
retinopathy consists of a salt-and-pepper pigmentary change or a mottled, blotchy, irregular
pigmentation, usually with the greatest density in the macula. The retinopathy is benign and
nonprogressive and does not interfere with vision (in contrast to the cataract) unless choroid
neovascularization develops in the macula.
Congenital heart disease including patent ductus arteriosus (PDA) and pulmonary artery
stenosis is present in 50% of infants infected in the first 2 months' gestation. Cardiac defects
and deafness occur in all infants infected during the first 10 weeks of pregnancy and deafness
alone is noted in one third of those infected at 13-16 weeks of gestation.

Other findings in congenital rubella syndrome include the following:

Intrauterine growth retardation, prematurity, stillbirth, and abortion

CNS abnormalities, including mental retardation, behavioral disorders, encephalographic
abnormalities, hypotonia, meningoencephalitis, and microcephaly
Hepatosplenomegaly
Jaundice
Hepatitis
Skin manifestations, including blueberry muffin spots that represent dermal erythropoiesis and
dermatoglyphic abnormalities
Bone lesions, such as radiographic lucencies
Endocrine disorders, including late manifestations in congenital rubella syndrome usually
occurring in the second or third decade of life (eg, thyroid abnormalities, diabetes mellitus)
Hematologic disorders, such as anemia and thrombocytopenic purpura

Table 2. Clinicopathologic Abnormalities in Congenital Rubella (Open Table in a new window)

Abnormality Common/Uncommon Early/Delayed Comment

General

Intrauterine growth
Common Early ...
retardation

Prematurity Uncommon Early ...

Stillbirth Uncommon Early ...

Abortion Uncommon Early ...

Cardiovascular system

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Patent ductus arteriosus Common Early May occur with

pulmonary artery
stenosis

Pulmonary artery Caused by intimal

Common Early
stenosis proliferation

Coarctation of the aorta Uncommon Early ...

Myocarditis Uncommon Early ...

Ventricular septal defect Uncommon Early ...

Atrial septal defect Uncommon Early ...

Eye

Cataract Common Early Unilateral or bilateral

Salt-and-pepper
appearance; visual
Retinopathy Common Early
acuity unaffected;
frequently unilateral

Cloudy cornea Uncommon Early Spontaneous resolution

Glaucoma Uncommon Early/Delayed May be bilateral

Common in patients
Microphthalmia Common Early
with unilateral cataract

Retinopathy with
Subretinal
Uncommon Delayed macular scarring and
neovascularization
loss of vision

Ear

Usually bilateral; mostly

sensorineural; may be
central in origin; rare
Hearing loss Common Early/Delayed when maternal rubella
occurs >4 months'
gestation; sometimes
progressive

CNS

Meningoencephalitis Uncommon Early Transient

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Microcephaly Uncommon Early May be associated with

normal intelligence

Intracranial calcifications Uncommon Early ...

Encephalographic Usually disappear by

Common Early
abnormalities age 1 y

Mental retardation Common Delayed ...

Frequently related to
Behavioral disorders Common Delayed
deafness

Autism Uncommon Delayed ...

Chronic progressive Manifest in second

Uncommon Delayed
panencephalitis decade of life

Hypotonia Uncommon Early Transitory defect

Uncommon in absence
Speech defects Common Delayed
of hearing loss

Skin

Represents dermal
Blueberry muffin spots Uncommon Early
erythropoiesis

Usually generalized;
Chronic rubelliform rash Uncommon Early
lasts several weeks

Dermatoglyphic
Common Early ...
abnormalities

Lungs

Generalized; probably
Interstitial pneumonia Uncommon Delayed immunologically
mediated

Liver

Hepatosplenomegaly Common Early Transient

Usually appears in the

Jaundice Uncommon Early
first day of life

May not be associated

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May not be associated
Hepatitis Uncommon Early with jaundice

Blood

Transient; no response
Thrombocytopenia Common Early
to steroid therapy

Anemia Uncommon Early Transient

Hemolytic anemia Uncommon Early Transient

Altered blood group

Uncommon Early ...
expression

Immune system

Hypogammaglobulinemia Uncommon Delayed Transient

Lymphadenopathy Uncommon Early Transient

Thymic hypoplasia Uncommon Early Fatal

Bone

Transient; most
Radiographic lucencies Common Early common in distal femur
and proximal tibia

Large anterior fontanel Uncommon Early ...

Micrognathia Uncommon Early ...

Endocrine glands

Usually becomes
Diabetes mellitus Common Delayed apparent in second or
third decade of life

Hypothyroidism,
Thyroid disease Uncommon Delayed hyperthyroidism, and
thyroiditis

Growth hormone
Uncommon Delayed ...
deficiency

Genitourinary system

Cryptorchidism Uncommon Early ...

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Cryptorchidism Uncommon Early ...

Polycystic kidney Uncommon Early ...

Causes
Rubella and congenital rubella syndrome are caused by rubella virus. Only one antigenic type of
rubella virus is available, and humans are the only natural hosts. The virus is spherical with a diameter
of 50-70 nm, has a central core (ie, nucleocapsid), and is covered externally by a lipid-containing
envelope. The nucleocapsid is composed of polypeptide (C protein) and a single-stranded RNA.

Its outer envelope is made up of glycosylated lipoprotein, which contains 2 virus-specific polypeptides
(E1, E2) and a host-cellderived lipid. These 2 envelope proteins comprise the spiked 5-nm to 6-nm
surface projections that are observed on the outer membrane of rubella virus and are important for the
virulence of the virus.

Monoclonal antibodies directed against epitopes of E1 and E2 have neutralizing activity. Protein E1 is
the viral hemagglutinin that binds both hemagglutination-inhibiting and hemolysis-inhibiting antibodies.

Rubella virus is rapidly inactivated by 70% alcohol, ethylene oxide, formalin, ether, acetone,
chloroform, free chlorine, deoxycholate, beta-propiolactone, ultraviolet light, extreme pH (< 6.8 or
>8.1), heat (>56C), and cold (from -10C to -20C). It is resistant to thimerosal and is stable at
temperatures of -60C or less.

Differential Diagnoses

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Media Gallery

Number of rubella cases per year.

Number of congenital rubella syndrome cases per year.
Deaths from rubella per year.
Image in a 4-year-old girl with a 4-day history of low-grade fever, symptoms of an upper
respiratory tract infection, and rash. Courtesy of Pamela L. Dyne, MD.
Number of rubella and congenital rubella syndrome (CRS) cases United States, 19662011.
Courtesy of Centers for Disease Control (CDC).

of 5

Tables

Table 1. Reported Cases of Rubella, Deaths From Rubella, and Number of Cases of Congenital
Rubella Syndrome in the United States From 1969-2007 [5, 6, 7, 8]
Table 2. Clinicopathologic Abnormalities in Congenital Rubella
Table 3. Age-Specific CD4+ T-lymphocyte Count and Percentage of Total Lymphocytes as a
Criteria for Severe Immunosuppression in Persons with HIV

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Table 1. Reported Cases of Rubella, Deaths From Rubella, and Number of Cases of Congenital
Rubella Syndrome in the United States From 1969-2007 [5, 6, 7, 8]

Year Number of Cases Number of Deaths Cases of Congenital Rubella Syndrome

1969 57,686 29 31

1970 56,552 31 77

1971 45,086 20 68

1972 25,507 14 42

1973 27,804 16 35

1974 11,917 15 45

1975 16,652 21 30

1976 12,491 12 30

1977 20,395 17 23

1978 18,269 10 30

1979 11,795 1 62

1980 3,904 1 50

1981 2,077 5 19

1982 2,325 4 7

1983 970 3 22

1984 752 1 5

1985 630 1 0

1986 551 1 5

1987 306 0 5

1988 225 1 6

1989 396 4 3

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1990 1,125 8 11

1991 1,401 1 47

1992 160 1 11

1993 192 0 5

1994 227 0 7

1995 128 1 6

1996 238 0 4

1997 181 0 5

1998 364 0 7

1999 267 0 9

2000 176 0 9

2001 23 2 3

2002 18 N/A 1

2003 7 N/A 1

2004 10 N/A 0

2005 11 N/A 1

2006 11 N/A 1

2007 12 N/A 0

Table 2. Clinicopathologic Abnormalities in Congenital Rubella

Abnormality Common/Uncommon Early/Delayed Comment

General

Intrauterine growth
Common Early ...
retardation

Prematurity Uncommon Early ...

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Prematurity Uncommon Early ...

Stillbirth Uncommon Early ...

Abortion Uncommon Early ...

Cardiovascular system

May occur with

Patent ductus arteriosus Common Early pulmonary artery
stenosis

Pulmonary artery Caused by intimal

Common Early
stenosis proliferation

Coarctation of the aorta Uncommon Early ...

Myocarditis Uncommon Early ...

Ventricular septal defect Uncommon Early ...

Atrial septal defect Uncommon Early ...

Eye

Cataract Common Early Unilateral or bilateral

Salt-and-pepper
appearance; visual
Retinopathy Common Early
acuity unaffected;
frequently unilateral

Cloudy cornea Uncommon Early Spontaneous resolution

Glaucoma Uncommon Early/Delayed May be bilateral

Common in patients
Microphthalmia Common Early
with unilateral cataract

Retinopathy with
Subretinal
Uncommon Delayed macular scarring and
neovascularization
loss of vision

Ear

Hearing loss Common Early/Delayed Usually bilateral; mostly

sensorineural; may be
central in origin; rare
when maternal rubella
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occurs >4 months'

gestation; sometimes
progressive

CNS

Meningoencephalitis Uncommon Early Transient

May be associated with

Microcephaly Uncommon Early
normal intelligence

Intracranial calcifications Uncommon Early ...

Encephalographic Usually disappear by

Common Early
abnormalities age 1 y

Mental retardation Common Delayed ...

Frequently related to
Behavioral disorders Common Delayed
deafness

Autism Uncommon Delayed ...

Chronic progressive Manifest in second

Uncommon Delayed
panencephalitis decade of life

Hypotonia Uncommon Early Transitory defect

Uncommon in absence
Speech defects Common Delayed
of hearing loss

Skin

Represents dermal
Blueberry muffin spots Uncommon Early
erythropoiesis

Usually generalized;
Chronic rubelliform rash Uncommon Early
lasts several weeks

Dermatoglyphic
Common Early ...
abnormalities

Lungs

Generalized; probably
Interstitial pneumonia Uncommon Delayed immunologically
mediated

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Liver

Hepatosplenomegaly Common Early Transient

Usually appears in the

Jaundice Uncommon Early
first day of life

May not be associated

Hepatitis Uncommon Early
with jaundice

Blood

Transient; no response
Thrombocytopenia Common Early
to steroid therapy

Anemia Uncommon Early Transient

Hemolytic anemia Uncommon Early Transient

Altered blood group

Uncommon Early ...
expression

Immune system

Hypogammaglobulinemia Uncommon Delayed Transient

Lymphadenopathy Uncommon Early Transient

Thymic hypoplasia Uncommon Early Fatal

Bone

Transient; most
Radiographic lucencies Common Early common in distal femur
and proximal tibia

Large anterior fontanel Uncommon Early ...

Micrognathia Uncommon Early ...

Endocrine glands

Usually becomes
Diabetes mellitus Common Delayed apparent in second or
third decade of life

Thyroid disease Uncommon Delayed Hypothyroidism,

hyperthyroidism, and
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hyperthyroidism, and
thyroiditis

Growth hormone
Uncommon Delayed ...
deficiency

Genitourinary system

Cryptorchidism Uncommon Early ...

Polycystic kidney Uncommon Early ...

Table 3. Age-Specific CD4 + T-lymphocyte Count and Percentage of Total Lymphocytes as a Criteria
for Severe Immunosuppression in Persons with HIV

Age
Range

< 12 mo 1-5 y 6-12 y 13 y

< < <

Total CD4+ T-lymphocytes < 750/mcl
500/mcl 200/mcl 200/mcl

CD4+ T-lymphocytes (as % of total < 15% < 15% < 15% < 14%
lymphocytes)

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Contributor Information and Disclosures

Author

Elias Ezike, MD Consulting Staff, Beaumont Pediatric Center, PLLC

Elias Ezike, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Coauthor(s)

Jocelyn Y Ang, MD, FAAP, FIDSA Associate Professor, Department of Pediatrics, Wayne State
University School of Medicine; Consulting Staff, Division of Infectious Diseases, Children's Hospital of
Michigan

Jocelyn Y Ang, MD, FAAP, FIDSA is a member of the following medical societies: American Academy
of Pediatrics, Infectious Diseases Society of America, Pediatric Infectious Diseases Society

http://emedicine.medscape.com/article/968523-clinical 16/17
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Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College
of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Russell W Steele, MD Clinical Professor, Tulane University School of Medicine; Staff Physician,
Ochsner Clinic Foundation

Russell W Steele, MD is a member of the following medical societies: American Academy of

Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for
Microbiology, Infectious Diseases Society of America, Louisiana State Medical Society, Pediatric
Infectious Diseases Society, Society for Pediatric Research, Southern Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

Leonard R Krilov, MD Chief of Pediatric Infectious Diseases and International Adoption, Vice Chair,
Department of Pediatrics, Winthrop University Hospital; Professor of Pediatrics, Stony Brook
University School of Medicine

Leonard R Krilov, MD is a member of the following medical societies: American Academy of

Pediatrics, American Pediatric Society, Infectious Diseases Society of America, Pediatric Infectious
Diseases Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Leslie L Barton, MD Professor Emerita of Pediatrics, University of Arizona College of Medicine

Leslie L Barton, MD is a member of the following medical societies: American Academy of Pediatrics,
Association of Pediatric Program Directors, Infectious Diseases Society of America, and Pediatric
Infectious Diseases Society

Disclosure: Nothing to disclose.

http://emedicine.medscape.com/article/968523-clinical 17/17