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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 22  |  Issue : 4  |  Page : 367-369

Congenital syphilis – Re-emergence of a much forgotten entity?


1 Department of Dermatology, Venereology and Leprosy, National Institute of Medical Sciences and Research, Jaipur, Rajasthan, India
2 Department of Skin & VD, Santokba Durlabhji Hospital, Jaipur, Rajasthan, India

Date of Submission09-Mar-2021
Date of Decision31-Mar-2021
Date of Acceptance01-Apr-2021
Date of Web Publication01-Oct-2021

Correspondence Address:
Sonal Jain
Department of Dermatology, Venereology and Leprosy, National Institute of Medical Sciences and Research, Jaipur - 303 121, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.ijpd_40_21

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  Abstract 


Congenital syphilis is a severe, disabling infection that occurs due to transmission of Treponema pallidum across the placenta. An increase in the incidence of congenital syphilis has been observed in developing countries. Here, we are reporting a case of early congenital syphilis who presented with desquamating papulosquamous lesions over multiple body parts along with moist erythematous and erosive lesions in the perianal and perioral regions. Reactive venereal disease research laboratory and T. pallidum hemagglutination assay test in child and parents confirmed the diagnosis of congenital syphilis.

Keywords: Congenital syphilis, Treponema pallidum hemagglutination assay, venereal disease research laboratory test


How to cite this article:
Amlani R, Agrawal S, Gulati R, Singh K, Jain S. Congenital syphilis – Re-emergence of a much forgotten entity?. Indian J Paediatr Dermatol 2021;22:367-9

How to cite this URL:
Amlani R, Agrawal S, Gulati R, Singh K, Jain S. Congenital syphilis – Re-emergence of a much forgotten entity?. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Dec 6];22:367-9. Available from: https://www.ijpd.in/text.asp?2021/22/4/367/327451




  Introduction Top


Sir William Osler aptly quoted” He who knows syphilis, knows medicine.”

Congenital syphilis was initially described in 1497 and is, therefore, the oldest recognized congenital infection.[1] It is acquired transplacentally from an infected mother to her fetus. In the recent years because of improved medical care, congenital syphilis has become uncommon.[2] In 2012, the WHO has estimated 350,000 adverse pregnancy outcome worldwide, as a consequence of syphilis. Adverse pregnancy outcome is 12 times more likely in women with syphilis than seronegative women, with 2–5-fold higher risk even after treatment as compared to uninfected women.[3] Approximately 10%–12% of infants born to mothers with positive serology for syphilis would die if untreated. We report a case of early congenital syphilis to emphasize that early congenital syphilis still occurs and proper antenatal screening is mandatory to prevent it.


  Case Report Top


A one-year-old male infant born out of nonconsanguineous marriage presented with skin lesions over extremities, perianal, oral, and perioral areas for the past 6 months. There was a history of fluid-filled lesions over legs and hands which ruptured spontaneously leaving behind raw areas. After 2 months, he developed similar asymptomatic lesions over tongue, oral cavity, perianal, perioral, and genital areas. There were no associated complaints such as diarrhea, runny nose, or failure to gain weight. The baby was delivered at full term by vaginal delivery with a birth weight of 2.8 kg. The child was asymptomatic at birth. Developmental milestones achieved were normal for the age.

Father had a history of extramarital sexual exposure. There was no history of abortion/still birth in mother.

Cutaneous examination revealed multiple well-defined erosions with dry erythematous base and scaling on the borders over bilateral palms, soles, dorsum of hands, feet, and knees [Figure 1]a, [Figure 1]b, [Figure 1]c. Multiple flat-topped moist erythematous plaques were present in the perianal and genital area [Figure 1]d. Split papules were seen on the left angle of mouth, and few well-defined erosions were also present on the dorsum of the tongue [Figure 1]e. Hair and nail were normal. There was no lymphadenopathy or hepatosplenomegaly. The patient was admitted in the pediatric department with differential diagnosis of early congenital syphilis and acrodermatitis enteropathica.
Figure 1: Pretreatment images. (a) Multiple well-defined erosions with dry erythematous base with scaling on bilateral hands. (b) Multiple well-defined erosions with dry erythematous base with scaling on bilateral soles. (c) A few well-defined erosions with dry erythematous base with scaling on bilateral knees. (d) Flat-topped moist erythematous plaques in the perianal area. (e) Well-defined erosions present on dorsum of the tongue with split papules on angle of mouth. (f) Marked cortical thickening and periosteal reaction noted in the lateral aspect of left tibia with saber shin deformity. (g) Osteitis of ribs in chest X-ray

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The general physical and systemic examination of patient, both parents and other sibling, was unremarkable.

The patient was subjected to venereal disease research laboratory (VDRL) test which revealed a dilution titer of 1:64 from blood and 1:5 from cerebrospinal fluid. Both parents had positive VDRL test with dilution titer of 1:128. VDRL in other sibling was negative. Other investigations are as shown in [Table 1].
Table 1: Investigations of the patient

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The clinicopathological correlation led to the diagnosis of early congenital syphilis.

The patient was treated with single dose of intramuscular injection benzathine penicillin (3.5 lac units) and injection ceftriaxone 250 mg IV twice daily for 14 days. The parents were treated with 3 weekly doses of intramuscular injection benzathine penicillin (2.4 million units) after sensitivity test.

The patient showed almost 90% improvement in skin lesions at the end of 14 days therapy which later cleared completely in 30 days [Figure 2]a, [Figure 2]b, [Figure 2]c. VDRL titer of the patient after 3 months was 1:16. The patient is under regular follow-up.
Figure 2: Posttreatment images. (a) Complete clearance of oral and perioral lesions posttreatment. (b) Near-complete healing of lesions of the sole posttreatment. (c) Near-complete clearance of condyloma lata lesions posttreatment

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  Discussion Top


Congenital Syphilis (syn. syphilis connata) occurs following transplacental transmission of Treponema pallidum to fetus in utero, which was first described in 1497.[4]

The term prenatal syphilis better indicates that the signs and symptoms may develop before or after delivery rather than always present at birth. It is divided into 2 subtypes [Figure 3].
Figure 3: Classification of congenital syphilis

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The risk of vertical transmission and fetal disease is related to stage of maternal syphilis during pregnancy.[5] Our case was asymptomatic at birth. About 60% of infants born with congenital syphilis are asymptomatic at birth.[6]

Mucocutaneous involvement is present in as many as 70% of infants and may be apparent at birth or develop during the firth few weeks of life. Early congenital syphilis manifests as vesiculobullous (syphilitic pemphigus) or maculopapular rash on palms and soles associated with desquamation.[7] Other findings of early congenital syphilis include fever, failure to thrive, hepatosplenomegaly, osteochondritis, pseudoparalysis, and rhinitis.[8] Our case presented with multiple papulosquamous lesions over palms, soles, extremities, and erythematous papules and plaques over perianal, genital, perioral, and oral areas. Other than skin our case presented with bony changes such as osteochondritis and saber tibia. Certain nutrient deficiencies such as zinc deficiency mimic skin rash of congenital syphilis. However, in our case, clinicopathological correlation led us to the diagnosis. Koh and Lim in a retrospective review of 13 cases observed osteochondritis and periostitis in patients.[4] In a large retrospective review of 302 clinically suspected cases of congenital syphilis, bone changes were found in 197, which included periostitis (52%), osteitis (15%), and metaphyseal changes (33%). More than one lesion in 36% of the cases were present.[9]

Abnormal laboratory parameters such as leukocytosis, Coombs negative hemolytic anemia, thrombocytopenia, hypoproteinemia, hypoalbuminemia, hyperbilirubinemia, and elevated liver enzyme may be present. Our patient had anemia and leukocytosis. The cause of anemia may be attributed to hemolysis, interference with hematopoiesis, and nutritional deficiency.[10]

As per Center for Disease Control and Prevention of sexually transmitted disease guidelines (2015), a proven or highly probable congenital syphilis is any neonate with[2] (1) an abnormal physical examination that is consistent with congenital syphilis or (2) a serum quantitative nontreponemal serologic titer that is four-fold higher than the mother's titer. (3) A positive dark-field test or PCR of lesion or body.

Recommended regimen in this scenario is aqueous crystalline penicillin G, 50,000 units/kg/dose IV every 12 h during the first 7 days and every 8 h thereafter for a total of 10 days or procaine penicillin G, 50,000 units/kg/dose in a single daily dose for 10 days.[11] However, due to the lack of availability of these penicillin preparations, injection benzathine penicillin and ceftriaxone were given in our case. Follow-up should be done at 2, 4, 6, 12, and 15 months.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Schmid GP, Stoner BP, Hawkes S, Broutet N. The need and plan for global elimination of congenital syphilis. Sex Transm Dis 2007;34:S5-10.  Back to cited text no. 1
    
2.
Modi M, Sharma A, Marfatia YS, Naik E, Toney J. Late congenital syphilis with stigmata. Indian J Sex Transm Dis 2008;29:32-3.  Back to cited text no. 2
  [Full text]  
3.
Gupta R, Vora RV. Congenital syphilis, still a reality. Indian J Sex Transm Dis 2013;34:50-2.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Koh MT, Lim CT. Early congenital syphilis: Experience with 13 consecutive cases seen at the University Hospital, Kuala Lumpur. Singapore Med J 1991;32:230-2.  Back to cited text no. 4
    
5.
Walker GJA, Walker DG. Congenital Syphilis: A continuing but neglected problem. Seminar in Fetal and Neonatal Medicine, 2007;12:198-206.  Back to cited text no. 5
    
6.
Waseem M, Aslam M. Pediatric Syphilis. Medscape Reference. Available from: http://www.emedicine.medscape.com/article /969023-overview. [Last accessed on 2018 Apr 18].  Back to cited text no. 6
    
7.
King A, Nicol C, Rodin P. Venereal Diseases. 4th ed. London: ELBS; 1980. p. 104-32.  Back to cited text no. 7
    
8.
Chakraborty R, Luck S. Syphilis is on the increase: The implications for child health. Arch Dis Child 2008;93:105-9.  Back to cited text no. 8
    
9.
Rasool MN, Govender S. The skeletal manifestations of congenital syphilis. A review of 197 cases. J Bone Joint Surg Br 1989;71:752-5.  Back to cited text no. 9
    
10.
Shah KH, Jagati AG, Rathod SP, Chaudhary RG. Early congenital syphilis: Resurgence of an entity nearing elimination. Indian J Paediatr Dermatol 2019;20:154-6.  Back to cited text no. 10
  [Full text]  
11.
cdc.gov. Atlanta: Centre for Disease Control and Prevention. Available from http://www.cdc.gov/std/treatment/[internet. [Last updated on 2015 Jun 05].  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

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