Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Net Case
doi: 10.4103/0378-6323.72475
PMID: 21079337

Fetal varicella syndrome

S Ramachandra, Arun Kumar Metta, Nayeem Sadath Haneef, Sandeep Kodali
 Department of Dermatology, Venereology and Leprology, Kamineni Institute of Medical Sciences, Narketpally, Nalgonda, Andhra Pradesh, India

Correspondence Address:
S Ramachandra
Professor and Head, Department of DVL, Kamineni Institute of Medical Sciences, Narketpally, Nalgonda, Andhra Pradesh
How to cite this article:
Ramachandra S, Metta AK, Haneef NS, Kodali S. Fetal varicella syndrome. Indian J Dermatol Venereol Leprol 2010;76:724
Copyright: (C)2010 Indian Journal of Dermatology, Venereology, and Leprology


Fetal varicella syndrome is a rare condition of the newborn, presenting with cutaneous scars, limb defects and ocular and central nervous system abnormalities. It is due to varicella or zoster developing in the fetus following maternal varicella infection during early pregnancy. We are reporting one such patient who presented with a linear, depressed, erythematous scar over the left forearm and axillary fold, with a history of maternal chicken pox during the first trimester of pregnancy.
Keywords: Fetal varicella syndrome, congenital varicella syndrome, congenital varicella zoster syndrome


Fetal varicella syndrome (FVS) is a rare complication of maternal varicella infection occurring in <2% of the babies born to women infected with varicella between 7 and 28 weeks of pregnancy. [1],[2],[3] The newborn may present with low birth weight, cutaneous scars, papular lesions, localized absence of skin on a limb, hypoplasia of one or more limbs, malformed digits along with various ocular and central nervous system (CNS) abnormalities. [3],[4] We are reporting one such case in view of paucity of reports from the Indian subcontinent. [5]

Case Report

Dermatologists opinion was sought for a newborn male baby on the 2 nd day of birth, born with scars over the left upper limb. There was no history of vesiculobullous lesions. The baby was born to a 22-year-old lady (gravida 2 para 1) at full term by cesarian delivery for non-progression of labor. She had a history of chicken pox during the first trimester of pregnancy. Birth weight of the child was 3.2 kg and APGAR score was 8-10. There was no history of consanguinity. Investigations like ultrasonography (fetal anomaly scan) or TORCH battery could not be performed as the mother did not get routine antenatal check ups in our hospital. The previous delivery was a full-term normal vaginal delivery and the baby was healthy.

Cutaneous examination of the child revealed a linear, irregular, depressed, erythematous scar measuring 12 cm × 5 cm over the anteromedial aspect of the left forearm. A similar small scar was present over the left axillary fold [Figure - 1] and [Figure - 2]. This distribution corresponds to C8 and T1 dermatomes. There was no limb hypoplasia, malformed digits, ocular changes or CNS abnormalities. There were no skeletal deformities on X-ray. The patient′s routine blood and urine parameters were within normal limits. His blood group was O positive and Venereal Diseases Research Laboratory test and human immunodeficiency virus antibodies were non-reactive. Differential diagnosis of neonatal scars was considered [Table - 1], but a diagnosis of FVS was made based on the characteristic history and clinical features.

Figure 1 :Irregular, depressed, erythematous scars over the left forearm and axillary fold
Figure 2 :Close-up view of the scar over the left forearm
Table 1 :Differential diagnosis of neonatal scars

The parents of the baby were counseled about the condition and reassured. No active intervention was advised at this stage.


Chicken pox may develop during pregnancy in one to five per 10,000 pregnancies. If infection occurs during the last 2 weeks of pregnancy, the infection may be transmitted to the fetus and the newborn may present with varicella at or soon after birth. [2] The neonate may develop severe disseminated varicella and septicemia with increased mortality (up to 28%) if maternal infection occurs 5 days before or after delivery due to the absence of protective maternal antibodies. [2] Occasionally, herpes zoster can occur in early infancy if the child has been infected with varicella zoster virus during early intrauterine life. [6] Maternal varicella occurring between 7 and 28 weeks of pregnancy may lead to either spontaneous abortions or "fetal varicella syndrome" [Table - 2]. [2],[4],[5],[6]

Table 2 :Gestational period-wise outcome of maternal chickenpox

FVS was first described in 1947 by La Foret and Lynch. [1] Srabstein et al. rediscovered the syndrome in 1974 and named it "congenital varicella syndrome." [7],[8] Subsequently, the condition has been reported under various names like congenital varicella zoster syndrome, fetal varicella zoster syndrome, varicella embryopathy, varicella embryofetopathy, etc.

A majority of the cases (90%) of FVS occur as a complication due to maternal varicella infection during the 1 st or 2 nd trimesters of pregnancy, and a few cases (10%) follow maternal herpes zoster. [7] Risk of transmission to fetus is approximately 2% and is especially more common when the mother has infection between the 13 th and 20 th weeks of gestation. [3],[6]

The typical clinical features include low birth weight, cutaneous lesions like scars often in a dermatomal outline (70%), papular lesions resembling connective tissue nevi, ocular abnormalities (66%) like choreoretinitis, cataracts, microophthalmia, Horner′s syndrome, nystagmus, limb hypoplasia (50%), CNS abnormalities (46%) including seizures, mental retardation, hydrocephalus, cortical atrophy, cerebellar aplasia, encephalomyelitis and dorsal radiculitis, and poor sphincter control (32%). [7]

In anticipated cases of FVS, amniocentesis, fetal blood and chorionic villous sampling may be performed to isolate the virus or to detect specific IgM. Pregnant women who are suspected not to be immune and who experience exposure to varicella zoster should be given varicella zoster immunoglobulin (VZIG) within 3 days after contact, although the efficacy is doubtful. [2] Termination of pregnancy is not indicated as the risk of fetal damage is less. Plastic surgery may be considered for the scars and limb abnormalities.

The case described above shows cutaneous scars typical of FVS, and there is history of varicella in the mother during the first trimester of pregnancy. The segmental distribution of scars probably reflects damage to the fetal nervous system by the neurotrophic virus. [8],[9] Our case is being reported to increase awareness about this rare but fascinating condition, which requires a high index of suspicion and careful history for diagnosis.

La Foret EG, Lynch LL. Multiple congenital defects following maternal varicella. N Eng J Med 1947;236:534-7.
[Google Scholar]
Criton S. Viral infections. In: Valia RG, Valia AR, editors. IADVL Textbook of dermatology. 3 rd ed. Mumbai: Bhalani Publishing House; 2008. p. 331-96.
[Google Scholar]
Pastuszak AL, Levy M, Schick B, Zuber C, Feldkamp M, Gladstone J, et al. Outcome after maternal varicella infection in the first 20 weeks of pregnancy. N Eng J Med 1994;330:901-5.
[Google Scholar]
Enders G, Miller E, Cradock-Watson J, Bolley I, Ridehalgh M. Consequences of varicella and herpes zoster in pregnancy: prospective study of 1739 cases. Lancet 1994;343:1548-51.
[Google Scholar]
Sasidharan CK, Anoop P. Congenital varicella syndrome. Indian J Pediatr 2003;70:101-3.
[Google Scholar]
Sterling JC. Virus infections. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's textbook of dermatology. 7 th ed. Oxford: Blackwell Science Ltd; 2004. p. 251-83.
th ed. Oxford: Blackwell Science Ltd; 2004. p. 251-83.'>[Google Scholar]
Gershon AA, Silverstein SJ. Varicella-zoster virus. Richman DD, Whitley RJ, Hayden FG, editors. Clinical virology. New York: Churchill Livingstone;1997. p. 421-44.
[Google Scholar]
Srabstein JC, Morris N, Larke RP, DeSa DJ, Castelino BB, Sum E. Is there a congenital varicella syndrome? J Paediatr 1974;84:239-43.
[Google Scholar]
Atherton DJ, Gennery AR, Cant AJ. The neonate. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's textbook of dermatology. 7 th ed. Oxford: Blackwell Science Ltd; 2004. p. 1.1-14.86.
th ed. Oxford: Blackwell Science Ltd; 2004. p. 1.1-14.86.'>[Google Scholar]

Fulltext Views

PDF downloads
Show Sections