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Year : 2016  |  Volume : 17  |  Issue : 2  |  Page : 151-152

Congenital varicella syndrome

1 Department of Dermatology, B. J. Medical College, Ahmedabad, Gujarat, India
2 Department of Dermatology, GMERS Medical College, Gandhinagar, Gujarat, India

Date of Web Publication30-Mar-2016

Correspondence Address:
Bela Padhiar
Plot No. 255/A, Sector 20, Gandhinagar - 382 020, Gujarat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.172472

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A 7 month old female presented with localized scarring over right buttock and right leg with hypoplasia of right lower limb. Mother had history of chicken pox at tenth week of pregnancy. We confirm the diagnosis of varicella zoster syndrome clinically.

Keywords: Congenital, syndrome, varicella

How to cite this article:
Chaudhary A, Padhiar B, Karia U, Parmar K, Shah B. Congenital varicella syndrome. Indian J Paediatr Dermatol 2016;17:151-2

How to cite this URL:
Chaudhary A, Padhiar B, Karia U, Parmar K, Shah B. Congenital varicella syndrome. Indian J Paediatr Dermatol [serial online] 2016 [cited 2022 Jul 7];17:151-2. Available from: https://www.ijpd.in/text.asp?2016/17/2/151/172472

  Introduction Top

Embryopathy characterized by limb atrophy and limb dermal scarring, due to maternal infection with varicella in the first or second trimester.

Congenital varicella syndrome is an extremely rare disorder in which affected infants have distinctive abnormalities at birth (congenital) due to the mother's infection with chickenpox (maternal varicella zoster) early during pregnancy (i.e. up to 20 weeks gestation). Affected newborns may have a low birth weight and characteristic abnormalities of the skin; the arms, legs, hands, and/or feet (extremities); the brain; the eyes; and/or, in rare cases, other areas of the body.

  Case Report Top

A 7-month-old female patient of consanguious marriage presented with scars over right leg since birth. There was no past history of vesiculobullous lesions. Mother had history of chickenpox during the first trimester (10th week) of pregnancy. The baby was born to a 25-year-old lady at full term by cesarian section delivery.

Cutaneous examination showed linear, irregular depressed erythematous scars over right leg and right buttock [Figure 1]. There were limb hypoplasia and absence of right little toe. Birth weight of the child was 2.2 kg. Hair and nails were normal. No central nervous system (CNS) and ocular changes.
Figure 1: Scarring present on right buttock and right leg

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  Investigations Top

Routine blood and urine parameters were within normal limits. IgM for varicella virus was normal.

Diagnosis was made by characteristic history and clinical features.

  Discussion Top

Maternal varicella in the first 20 weeks of pregnancy is associated with an approximate 2% risk of fetal damage, including skin lesions, CNS, ocular defects and limb hypoplasia with a 30% mortality within the 1st year of life.[1]

Sarring over sacral region and right lower leg [Figure 2]. Scarring over right thigh and leg [Figure 3]. We reported a 7-month-old female patient with localized scarring over right buttock and right leg with hypoplasia of right lower limb with the maternal history of chickenpox at 10th week of pregnancy. If a child is born with full blown congenital varicella syndrome, the mortality is around 25% in the first 3 months of life.[2]
Figure 2: Scarring over sacral region and right lower limb with limb hypoplasia and absence of little toe

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Figure 3: Scarring over right thigh and leg

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Pregnant woman who are not immune and who experience exposure to varicella zoster should be given varicella zoster immunoglobulin.[3]

  References Top

Sauerbrei A, Wutzler P. The congenital varicella syndrome. J Perinatol 2000;20:548-54.  Back to cited text no. 1
Sauerbrei A, Wutzler P. Herpes simplex and varicella-zoster virus infections during pregnancy: Current concepts of prevention, diagnosis and therapy. Part 1: Herpes simplex virus infections. Med Microbiol Immunol 2007;196:89-94.  Back to cited text no. 2
Prober CG, Gershon AA, Grose C, McCracken GH Jr, Nelson JD. Consensus: Varicella-zoster infections in pregnancy and the perinatal period. Pediatr Infect Dis J 1990;9:865-9.  Back to cited text no. 3


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
Nirmala Cherukuri,Swapna Lingaldinna,Sadiqua Anjum,Himabindu Singh
Indian Journal of Child Health. 2019; 6(3): 142
[Pubmed] | [DOI]


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