An Infant with Seizures, Rash, and Hepatosplenomegaly

Diagnosis: congenital syphilis. The infant's rapid plasma reagin test was reactive at a titer of 1:256 dilutions; a Treponema pallidum particle agglutination assay and fluorescent treponemal antibody absorption test were also reactive. A CSF sample that was obtained after the patient had received 4 days of antibiotics revealed a WBC count of 6 cells/mm3 (7% polymorphonuclear cells, 70% lymphocytes, and 23% monocytes and macrophages), an RBC count of 32 cells/mm3, a protein level of 0.43 g/L, and a glucose level of 2.2 mmol/L; a Venereal Disease Research Laboratory test was nonreactive. Long-bone radiographs and MRIs of the brain had normal findings. The patient was treated with penicillin G for 10 days. The rash (figures 1–3) had improved markedly at the time of hospital discharge, but the patient was still requiring supplemental nasogastric feeds because of a poor suck. A follow-up rapid plasma reagin test that was performed 3 months after initiation of therapy was reactive at a titer of 1:8 dilutions. Scaly, erythematous erosions with collaretes of crust and numerous bullae covering the entire lower limbs of the neonate. Scalp of the infant, showing crusted erythematous erosion following a ruptured bullae. Pemphigoid-like lesions with superficial desquamation, which sometimes follows rupturing of a bullae. The mother reported a history of having a macular rash on her trunk, upper extremities, and the dorsum of her hands 1 month postpartum. During her child's hospitalization, the mother had a rapid plasma reagin test that was reactive at 1:128 dilutions, as well as a reactive T. pallidum particle agglutination assay and fluorescent treponemal antibody absorption test; she received a diagnosis of secondary syphilis, with her infection clearly having been acquired during pregnancy. Approximately 60% of infants with congenital syphilis are asymptomatic at birth [1]. Early manifestations typically occur at 2–13 weeks of age but can occur at up to 2 years of age, with more than one-half of cases involving a rash [2, 3]. Dermatologic manifestations range from desquamation alone to a generalized maculopapular rash (often presenting as bright red, raised, palpable lesions that gradually fade), mucous patches, papulosquamous lesions (especially on the palms and soles), or vesicobullous lesions. Syphilitic pemphigus is a term that refers to an increasingly rare and uniquely neonatal vesicobullous rash that is located primarily on the palms and soles and consists of dark red papules that evolve into bullae teeming with treponemes; the bullae are 1–5 cm in diameter with a red indurated base and leave a macerated brown crust following bullae rupture [2]. In a consecutive survey of 206 cases of congenital syphilis, none of the patients had syphilitic pemphigus [3]. In our case, because material from the bullae was not tested for treponemes, it is difficult to be certain whether the bullae constituted true syphilitic pemphigus. There was concern that the patient's hypoglycemia and hyponatremia could be indicative of syphilitic hypopituitarism, but an elevated random serum cortisol level excluded this diagnosis, and the etiology of the hypoglycemia was presumably poor feeding. It is possible that our patient had hyponatremia caused by intersititial nephritis, but nephrotic syndrome is a much more common renal manifestation of congenital syphilis [4]. There has been a resurgence of syphilis in the province of Alberta, Canada, with 9 cases of congenital syphilis in 2005 and 2006 (including our case) after no cases during 1992–2002 [5]. Universal prenatal serologic screening for syphilis plays a crucial role in the prevention of vertical transmission of syphilis. As was demonstrated in this case, universal rescreening of all pregnant women in areas experiencing outbreaks of infectious syphilis is valuable, because women with no apparent risk factors can acquire syphilis during pregnancy. However, even if such rescreening occurs, the sensitivity of the rapid plasma reagin test is estimated to be only 62%–76% in early primary syphilis [6], and more sophisticated methods of screening, such as the EIA for syphilis, may be required to detect all cases [7]. Potential conflicts of interest.All authors: no conflicts.

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