Indian Journal of Paediatric Dermatology

CASE REPORT
Year
: 2019  |  Volume : 20  |  Issue : 2  |  Page : 154--156

Early congenital syphilis: Resurgence of an entity nearing elimination


Khushali H Shah, Ashish G Jagati, Santoshdev P Rathod, Raju G Chaudhary 
 Department of Dermatology, V S General Hospital, Ahmedabad, Gujarat, India

Correspondence Address:
Dr. Khushali H Shah
Department of Dermatology, V S General Hospital, Near Ellis Bridge, Ahmedabad - 380 006, Gujarat
India

Abstract

A 45-day-old female infant was brought to a tertiary care center with the chief complaints of skin lesions over both hands and feet and perianal area since birth. Associated systemic complaints included rhinitis and diarrhea. Clinical examination revealed erosions with moist, erythematous base in an acral distribution, cheilitis, and perianal moist erythematous lesion. Based on the history and clinical examination findings of a congenital bullous rash, acrodermatitis, and periorificial moist lesion, rapid plasma reagin test was advised which came 1:512, thus confirming the diagnosis of congenital syphilis.



How to cite this article:
Shah KH, Jagati AG, Rathod SP, Chaudhary RG. Early congenital syphilis: Resurgence of an entity nearing elimination.Indian J Paediatr Dermatol 2019;20:154-156


How to cite this URL:
Shah KH, Jagati AG, Rathod SP, Chaudhary RG. Early congenital syphilis: Resurgence of an entity nearing elimination. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Sep 21 ];20:154-156
Available from: http://www.ijpd.in/text.asp?2019/20/2/154/255208


Full Text



 Introduction



Congenital syphilis, a burning disease of the past, is now a disease considered as one, nearing elimination, but resurfacing at occasional intervals with typical and atypical clinical presentations. The decreasing incidence and the associated stigmata and long-term sequelae prompt it to be one of the cases which draw attention to their resurgence. We hereby report a case of early congenital syphilis which presented to our outpatient department.

 Case Report



A 45-day-old female patient born to a 24-year-old mother and 28-year-old father, by a full-term, vaginal delivery, with a birth weight of 1.7 kg presented with skin lesions over palms, soles, and perianal region since birth. There was a history of fluid-filled lesions over legs and hands which ruptured spontaneously to leave behind raw areas. Associated complaints as noted by the mother included diarrhea, running nose, and a failure to gain weight. Records of antenatal visits were not available. There was a history of self-healing, asymptomatic genital erosion in father, no history of skin lesions in the sibling or mother, but examination revealed asymptomatic skin lesions over the palms of mother. The father had a history of multiple, paid and unpaid, homosexual and heterosexual exposure, and last exposure being 7 days ago, with a known female partner. Mother denied any history of sexual exposure.

Cutaneous examination of the patient revealed multiple erosions with a dry, erythematous base and scaling over borders of bilateral palms and soles [Figure 1]a and [Figure 1]b. Erythematous, bilaterally symmetrical, moist patches were present over the perianal area [Figure 1]c. Cheilitis with a moist erosion over the upper lip was present [Figure 1]d. The presence of a mild hepatomegaly was noted. There was excessive crying along with an aggravated irritability on touching the patient, which raised the suspicion of pseudoparalysis. Conjunctival mucosa, hair, nails, and lymph nodes were normal. Developmental milestones – head holding – were achieved along with a normal breastfeeding reflex.{Figure 1}

Examination of mother revealed few discrete, skin-colored papulosquamous lesions in both the palms; mucosa and lymph nodes being normal.

Examination of father revealed two–three nontender, firm, shotty, discrete, bilaterally palpable inguinal lymph nodes with overlying skin normal, without any other cutaneous findings such as a healed scar or postinflammatory hypopigmentation over the genitals.

The patient and her parents were subjected to rapid plasma regain (RPR) test, which revealed a 1:512 titer in the patient and 1:32 titer in both the parents, thus confirming the diagnosis of early congenital syphilis in the patient. The patient was admitted under the pediatrics department with combined care of dermatology, and routine investigations as well as cerebrospinal fluid analysis were done [Table 1], [Figure 2]. Ophthalmological examination showed no abnormalities.{Table 1}{Figure 2}

The patient was started on aqueous crystalline penicillin G 100,000 international units intravenous every six hours for 14 days and parents were treated with injection benzathine penicillin (2.4 million units), three intramuscular injections at weekly interval, after a negative test dose, as per the standard treatment guidelines. The skin lesions of the patient resolved by 7–10 days, and that of the mother by 1 week after the first dose of penicillin and steady improvement in the general status of the patient were noted as evidenced by a posttreatment weight of the patient being 2.4 kg and a decrease in irritability.

 Discussion



Congenital syphilis is a rare cause of neurological, developmental, and musculoskeletal disability and death in infants, after the advent of penicillin. The resource-poor, infected infants may experience severe sequelae which can be prevented by timely treatment of prenatal syphilis during pregnancy. Congenital syphilis occurs when Treponema pallidum crosses the placenta from the mother to the fetus during pregnancy or by contact with an infectious lesion during birth.[1] Diagnosis of early congenital syphilis is difficult because more than half of the infants are asymptomatic, and signs in symptomatic infants may be subtle and nonspecific. Manifestations of congenital syphilis are divided into early and late signs based on the first two years of life. Mucocutaneous involvement is present in as many as 70% of infants and may be apparent at birth or develop during the first few weeks of life. Cutaneous findings of early congenital syphilis are classically a vesiculobullous (sometimes called the syphilitic pemphigus) or maculopapular rash on the palms and soles and may be associated with desquamation.[2] Other types of rashes such as erythema multiforme have also been reported.[2] In addition, symptoms of early congenital syphilis include fever, failure to thrive, hepatosplenomegaly, lymphadenopathy, osteochondritis, pneumonitis, pseudoparalysis, and rhinitis.[3] Rhinitis (“snuffles”) has been reported in as many as 73% of infants. It usually develops in the 2nd–3rd week of life and may be the earliest clinical sign.[4] In our patient, rhinitis, periorificial dermatitis, papular acrodermatitis, and a history of vesiculobullous lesions since birth were the cardinal cutaneous features which led us to suspect early congenital syphilis.

Although acral dermatitis and vitamin or nutrient deficiency might mimic this disorder, skin rash of early congenital syphilis is relatively recalcitrant to treatment, which might be differential diagnostic point. Characteristic mucocutaneous rash, presenting with erythematous maculopapular or bullous lesions, followed by desquamation involving hands and feet, is common in congenital syphilis.

In terms of laboratory parameters, leukocytosis, Coombs-negative hemolytic anemia, thrombocytopenia, hypoproteinemia, hypoalbuminemia, hyperbilirubinemia, and elevated liver enzyme levels may be present. Anemia has been found to be associated with early congenital syphilis. There is no definite known cause of anemia, but it may be attributed to hemolysis, interference with hematopoiesis, hypersplenism, and nutritional deficiency. Thrombocytopenia, in association with hemolytic anemia, was commonly present early in the disease, often before the “classical” signs of syphilis developed. The cause of thrombocytopenia is postulated due to decrease in platelet survival, rather than a decrease in marrow production. Jaundice is characterized by elevations of both conjugated and unconjugated bilirubin. Because these laboratory findings are difficult to identify upon physical examination, a high index of suspicion is necessary to make the right diagnosis early.

In our patient, the laboratory abnormalities of a positive RPR test, anemia, raised alkaline phosphatase level, and X-ray findings further supported the clinical diagnosis of early congenital syphilis. Furthermore, a moderately raised cerebrospinal fluid (CSF) protein and lymphocyte value along with a weakly positive CSF RPR in our patient also correlated with the “Type 2” CSF,[2] leading to a diagnosis of asymptomatic neurosyphilis. Loss of density at the medial aspect of tibia, known as the Wimberger sign, was also appreciated in our patient.

As per the standard treatment guidelines, all infants fulfilling any one of the following criteria should receive treatment.[5]

Whose RPR/venereal disease research laboratory (VDRL) titer is four-fold higher than that of the mother at deliveryBorn to mothers with clinical evidence of syphilisBorn to mothers whose RPR/VDRL titer did not drop four-fold

For infants who are at least 4 weeks of age or older children, treatment is aqueous penicillin G, 50,000 units/kg/dose every six hours intravenously for 10–14 days because of the difficulty in excluding neurosyphilis in this age group.[6]

Follow-up should be done at 2, 4, 6, 12, and 15 months posttreatment.

To conclude, even in the era of increased social awareness regarding venereal diseases, the diagnosis of an old disease like congenital syphilis which is now uncommon should not be precluded.

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

1Woods CR. Syphilis in children: Congenital and acquired. Semin Pediatr Infect Dis 2005;16:245-57.
2King A, Nicol C, Rodin P. Venereal Diseases. 4th ed. London: ELBS; 1980. p. 104-32.
3Chakraborty R, Luck S. Syphilis is on the increase: The implications for child health. Arch Dis Child 2008;93:105-9.
4Rathod S, Shah B. Early prenatal syphilis. Indian Dermatol Online J 2010;1:39-41.
5Marfatia YS, Pandya I, Jose SK. Congenital syphilis: Marching toward elimination. Indian J Sex Transm Dis 2015;36:1-2.
6Arnold SR, Ford-Jones EL. Congenital syphilis: A guide to diagnosis and management. Paediatr Child Health 2000;5:463-9.