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CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 5  |  Page : 615-616
Congenital varicella syndrome with isolated limb hypoplasia and scarring: A rare fascinating case


Department of Dermatology, Venereology and Leprosy, Tirunelveli Medical College and Hospital, Tirunelveli, Tamil Nadu, India

Date of Web Publication29-Dec-2022

Correspondence Address:
Nirmaladevi Palanivel
Department of Dermatology, Venereology and Leprosy, Tirunelveli Medical College and Hospital, Tirunelveli, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_910_20

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How to cite this article:
Sethumadhavan S, Maalik Babu A N, Palanivel N, Baskar A. Congenital varicella syndrome with isolated limb hypoplasia and scarring: A rare fascinating case. Indian J Dermatol 2022;67:615-6

How to cite this URL:
Sethumadhavan S, Maalik Babu A N, Palanivel N, Baskar A. Congenital varicella syndrome with isolated limb hypoplasia and scarring: A rare fascinating case. Indian J Dermatol [serial online] 2022 [cited 2023 Feb 5];67:615-6. Available from: https://www.e-ijd.org/text.asp?2022/67/5/615/366142




Sir,

Congenital or fetal varicella syndrome (FVS) affects <2% of babies born to mothers infected with varicella between 7 and 28 weeks of pregnancy, especially during 13 to 20 weeks.[1] It classically presents with scars in a dermatomal distribution, limb hypoplasia, low birth weight, ocular and central nervous system abnormalities with mortality of 10% to 30%.[2] Although many cases of classical FVS with other systemic anomalies have been reported worldwide, but only few cases with isolated cutaneous manifestations and limb hypoplasia were reported.[3] We are reporting one such case without any ocular or systemic anomalies.

A 3-day-old low birth weight, severe intrauterine growth restriction (weight <third centile) male baby (38 weeks, 1.87 kg) born to a 27-year-old primi mother was referred to our hospital for neonatal intensive care unit. Baby was delivered by lower (uterine) segment caesarean section with APGAR (Appearance, Pulse, Grimace, Activity, and Respiration) score of 8/10. Baby was admitted for respiratory distress, suspected sepsis, jaundice and skin lesions. On examination, there was left upper limb hypoplasia [Figure 1] with malformed hypoplastic fingers [Figure 2]. A linear, irregular, atrophic scar measuring 8 × 2 cm was extending over the lateral aspect of the left lower half of arm, elbow and upper forearm (C5-C6 dermatome) associated with cicatricial erythematous linear scar 1 × 0.5 cm bridging the left lower arm and upper forearm across the lateral aspect of elbow [Figure 3], leading to a fixed flexion deformity of 30 degrees at elbow joint. Head circumference was normal (33 cm). Neurological and ophthalmological examinations were normal. As the skin lesions were consistent with FVS, maternal history was elicited, which revealed a history of chickenpox at 12 weeks of gestation for which no treatment was sought. Immunoglobulin (Ig) M ELISA (enzyme-linked immunoassay) for varicella-zoster virus was negative (1.79 U/mL) and IgG ELISA was strongly positive (>150 U/mL; normal: <8 U/mL). Ultrasound of abdomen, pelvis and cranium and X-ray of the chest were normal. Baby was discharged on breastfeeds at 24 days of life after recovering from sepsis, but he expired within 3 days at home.
Figure 1: Congenital varicella syndrome baby with left upper limb hypoplasia

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Figure 2: Same baby with malformed, hypoplastic, rudimentary digits in left hand

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Figure 3: Linear, irregular, atrophic scar over the lateral aspect of the left lower half of arm, elbow and upper two thirds forearm associated with bridging erythematous cicatricial scar across elbow

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Criteria to confirm the diagnosis of FVS include the following:

  • Clinical with virologic or serologic evidence of maternal varicella infection
  • Erosions or scars in a dermatomal distribution in the baby and
  • Immunologic evidence of varicella infection in the infant, either IgM antibody or persistence of IgG antibody beyond 1 year of life in the absence of clinical varicella infection.[1]


In this neonate, IgM was negative and IgG could not be repeated as baby expired. So we applied criteria proposed by Harger et al.[4] and a diagnosis of definitive FVS was made as it fulfilled the criteria, that is typical scarring and limb hypoplasia. The above-mentioned criteria could be used as an alternative to diagnose FVS as many of the infants expire before 1 year of age. FVS can be prevented by vaccination of the women in childhood or at least 1 month before conception. Administering varicella-zoster virus immunoglobulin to susceptible mothers, within 48 to 96 hours of contact with an infected person and early acyclovir to the infected pregnant mother may prevent or decrease the severity of FVS.[5]

Declaration of patient consent

The patient's parents gave their full permission for the publication, reproduction or other use of photographs. They understand that patient's name and initial will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Hunt RD, Friedlander SF. Viral infections. In: Eichenfield LF, editor. Neonatal and Infant Dermatology. 3rd ed. London: Elsevier Publishers; 2015. p. 182-6.  Back to cited text no. 1
    
2.
Mandelbrot L. Fetal varicella—diagnosis, management, and outcome. Prenat Diagn 2012;32:511-8.  Back to cited text no. 2
    
3.
Mehta S, Schenk W, Kirker S, Atrey A. Isolated lower limb hypoplasia secondary to congenital varicella syndrome: A rare occurrence and management of its complications. BMJ Case Rep 2017;2017:bcr2016218521.  Back to cited text no. 3
    
4.
Harger JH, Ernest JM, Thurnau GR, Moawad A, Thom E, Landon MB, et al. Frequency of congenital varicella syndrome in a prospective cohort of 347 pregnant women. Obstet Gynecol 2002;100:260-5.  Back to cited text no. 4
    
5.
Sauerbrei A. Preventing congenital varicella syndrome with immunization. CMAJ 2011;183:E169-70.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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