Desquamation of Infants’ Hands and Feet: An Uncommon Presentation of Congenital Syphilis
Desquamation of the palms and soles, such as that seen in the two infants’ cases described here, is a known but uncommon presentation of congenital syphilis.
Case 1
The patient is a 33-week-old girl who was born preterm via emergency cesarean delivery for nonreassuring heart tones. She was born to a gravida 2, para 1 mother who had had no prenatal care and who was an intravenous drug user who tested positive for amphetamines and cannabinoids on admission. The infant was admitted to the neonatal intensive care unit (NICU) for hydrops and respiratory distress.
Abnormal physical examination findings were dysmorphic facies, scalp edema, a grade 2/6 systolic murmur, desquamation of the palms and soles (Figures 1 and 2), hepatomegaly 5 cm below the costal margin, and splenomegaly 4 cm below the costal margin (Figure 3).
Results of screening laboratory tests showed the infant to have thrombocytopenia, anemia, elevated liver function test results, and cholestasis evidenced by an elevated γ-glutamyltranferase level.
The patient and her mother were tested for a multitude of infectious etiologies, and rapid plasma reagin (RPR) titers returned positive for syphilis at 1:16 in the neonate and 1:32 in mother. The mother stated that she had never been diagnosed with or treated for syphilis previously.
Confirmatory fluorescent treponemal antibody absorption (FTA-ABS) test results were positive for syphilis in the mother and the child; however, lumbar puncture was unable to be performed for a VDRL test in the infant. Results of a long bone radiographic survey was negative for skeletal involvement, and ophthalmologic examination findings were negative for eye involvement.
The infant was treated with intravenous penicillin G for 14 days, since cerebrospinal fluid (CSC) could not be obtained and thus central nervous system involvement could not be ruled out.
Case 2
A newborn boy was delivered at 34 weeks of gestation to a 23-year-old gravida 2, para 0 mother who had had limited prenatal care but no significant medical history. Upon admission of the neonate to the NICU, maternal RPR results were positive for syphilis, with a titer of 1:32. On further questioning, the mother stated that she had been aware of this diagnosis and had been treated with 1 dose of penicillin approximately 1 month previously.
On physical examination, the neonate had normal vital signs and was generally well appearing except for desquamation of the palms and soles (Figure 4 and 5). Additionally, he had hepatomegaly 2 cm below the costal margin and splenomegaly 1.5 cm below the costal margin. The rest of the physical examination findings were normal. Laboratory test results showed mild anemia and thrombocytopenia but were otherwise within normal limits.
Confirmatory FTA-ABS test results were positive for syphilis in both the mother and child; neonatal RPR results were positive with a titer of 1:32, while VDRL test results of the child’s CSF were negative. The patient received a diagnosis of congenital syphilis and completed a 10-day course of intravenous penicillin G.
Overview of Congenital Syphilis
Congenital syphilis is caused by a spirochete bacterium, Treponema pallidum. Syphilis is sexually transmitted to the mother and then vertically transmitted to her child during pregnancy. Testing for syphilis is the standard of care at prenatal visits; however, the infection goes undiscovered in many mothers as a result of limited prenatal care.
Although the prevalence of syphilis has decreased over the past century, an estimated 1.4 million pregnant women worldwide still are diagnosed with active syphilis every year.1
Syphilis is a treatable condition if it is diagnosed early, but significant morbidity and mortality still are possible, especially in untreated cases, including stillbirth, hydrops, prematurity, neonatal death, neurosyphilis, meningitis, blindness, and hearing loss.2
The diagnosis is made with positive results on maternal screening blood tests (VDRL or RPR), after which a confirmatory FTA-ABS or microhemagglutination assay for T pallidum antibodies (MHA-TP) is performed on the mother’s serum. If these test results are shown to be positive, then the infant requires syphilis testing.
Infant blood tests include a quantitative RPR or VDRL for an initial titer; a complete blood count to assess for anemia, thrombocytopenia, and leukopenia; and a comprehensive metabolic panel to assess for liver and renal effects. Additionally, a lumbar puncture must be performed for VDRL testing of CSF, as well as a long bone radiographic survey to assess for periosteal changes.3
A full physical examination should be completed, although infants with congenital syphilis infection often are asymptomatic at birth. Classic but rare examination findings can include skin lesions, hepatosplenomegaly, nasal discharge, pseudoparalysis, condylomata lata, pneumonia alba, and ophthalmologic findings.
Open skin lesions and areas of desquamation contain treponemal antigen; thus, contact precautions should be taken until the patient has been tested and treated.
Treatment of Congenital Syphilis
Infants requiring syphilis treatment include those born to a mother with untreated syphilis or who had been treated for syphilis less than 1 month prior to delivery; those whose mother has serologic evidence of relapse of infection or who had been treated for syphilis with a nonpenicillin regimen; those whose mother had poor serologic follow-up to treatment (a fourfold decrease in titer is required); or those who are symptomatic.
The preferred treatment of neonates with syphilis is intravenous aqueous crystalline penicillin G. Infants are treated with 50,000 units/kg/dose twice daily for infants up to 7 days old, then 50,000 units/kg/dose every 8 hours for the remaining days, for a total of 10 to 14 days of treatment.4,5 RPR titers should be assessed every 2 to 3 months until a fourfold decrease has occurred. If treated appropriately, these titers should continue to trend downward and should be nonreactive by 12 months of age.
Alessandra Guiner, MD, is a resident in the Department of Pediatrics at the University of Nevada School of Medicine in Las Vegas, Nevada.
Pisespong Patamasucon, MD, is a pediatric infectious disease specialist and a professor in the Department of Pediatrics at the University of Nevada School of Medicine in Las Vegas, Nevada.
References
1. Newman L, Kamb M, Hawkes S, et al. Global estimates of syphilis in pregnancy and associated adverse outcomes: analysis of multinational antenatal surveillance data. PLoS Med. 2013;10(2):e1001396.
2. Gomez GB, Kamb ML, Newman LM, Mark J, Broutet N, Hawkes SJ. Untreated maternal syphilis and adverse outcomes of pregnancy: a systematic review and meta-analysis. Bull World Health Organ. 2013;91(3):217-226.
3. Arnold S, Ford-Jones EL. Congenital syphilis: a guide to diagnosis and management. Paediatr Child Health. 2000;5(8):463-469.
4. Singh AE, Romanowski B. Syphilis: review with emphasis on clinical, epidemiologic, and some biologic features. Clin Microbiol Rev. 1999;12(2):187-209.
5. Workowski KA, Berman S. Sexually transmitted diseases treatment guidelines, 2010. MMWR Recomm Rep. 2010;59(RR-12):1-110.