|Year : 2015 | Volume
| Issue : 2 | Page : 75-77
Early congenital syphilis
Gnaneshwar Rao Angoori
Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India
|Date of Web Publication||9-Apr-2015|
Gnaneshwar Rao Angoori
F12, B8 HIG-2, APHB, Baghlingampally, Hyderabad - 500 044, Telangana
Source of Support: None, Conflict of Interest: None
Syphilis is a well-known cause of morbidity and mortality since ages. Significant increase in the incidence of congenital syphilis (CS) has been observed in developing countries. Herein, we report a case of CS in 1-month-old baby, who was referred for erythematous maculo-papular rash with desquamation. Serology of baby and mother for venereal disease research laboratory and treponema pallidum hamagglutination assay confirmed the diagnosis of CS.
Keywords: Congenital syphilis, maculo-papular rash, treponema pallidum hemagglutination assay, venereal disease research laboratory
|How to cite this article:|
Angoori GR. Early congenital syphilis. Indian J Paediatr Dermatol 2015;16:75-7
| Introduction|| |
Congenital syphilis (CS) has been known to man as early as 15 th century. Although the rate of CS is declining in developed countries, a significant increase has been observed in the under developed countries despite wide spread use of penicillin to treat syphilis since the early 1950s.  CS is mainly a due to lack of antenatal care and control of sexually transmitted infections. Proper antenatal syphilis screening is of primary importance to identify serum-positive women and to assure adequate treatment to prevent the risk of vertical transmission. 
| Case report|| |
A 1-month-old female baby was admitted with a history of refusal of feeds, distension of abdomen and ulcer over the left heel of 2-day duration. The baby was ill looking and icteric. Cutaneous examination revealed erythematous maculo-papular rash, with desquamation involving face, chest, upper limbs and lower limbs [Figure 1]. Hemorrhagic crust was noted on the left heel [Figure 2]. Cervical lymph nodes on both sides were enlarged, discrete and nontender. Palms and soles showed scaling and fissuring. Mucosae were normal. Ophthalmic and otological examination was normal. With these features, provisional diagnosis of viral exanthema was made. However, miliaria crystallina, CS, and seborrheic dermatitis were considered in the differential diagnosis. On investigation, she was found to be thrombocytopenic (platelet count 40,000/cmm) with elevated serum bilirubin (total bilirubin 12 mg%). Hepatomegaly distended gall bladder and absence of the left kidney were noted on ultrasonography. X-ray of long bones showed evidence of metaphyseal bands, metaphysitis, and periosteitis [Figure 3]. Venereal disease research laboratory (VDRL) test (1:64 dilutions) and Treponema pallidum maggultination assay (TPHA) 1:2560 dilution were positive. Baby's mother was positive for TPHA (1:2560) and VDRL test (1:32). Serology of both mother and baby was negative for HIV 1 and 2. Patient's first sibling was nonreactive for VDRL and TPHA. Positive serological tests for syphilis in both mother and baby and characteristic radiological changes along with a characteristic rash in the baby pointed to the diagnosis of CS. The baby was put on injection crystalline penicillin 50,000 IU/kg 8 th hourly for 10 days. However, during the course of treatment she developed bronchopneumonia, to which she succumbed. Her parents were treated with a single dose of benzathine penicillin 2.4 mega units.
|Figure 1: Diffuse erythematous maculo-papular rash with scaling involving face, upper chest, upper limbs|
Click here to view
|Figure 2: Scaling involving both soles with hemorrhagic crust on the left heel|
Click here to view
|Figure 3: Radiography shows metaphysitis and periostitis of long bones of upper and lower limbs|
Click here to view
| Discussion|| |
Congenital syphilis occurs due to transplacental transmission of spirochetes from an asymptomatic infected mother to an infant, was first described in 1906.  Untreated pregnant women with primary and secondary syphilis with consequential spirochetemia are more likely to transmit infection than women with latent infection. Transmission can occur throughout pregnancy, and 40% of affected fetuses die in intrauterine or perinatal period.  The risk of vertical transmission and fetal diseases are directly related to the stage of maternal syphilis during pregnancy. It is estimated that in women with syphilis of a few years duration, about half of the pregnancies will be affected, with one half of the affected pregnancies ending in stillbirth (including miscarriages), and the other half in perinatal death or serious neonatal infection (CS).  The risk of fetal loss and CS drops slightly in early latent stage and decreases to 10% in late latent stage, respectively. 
Clinical manifestations of CS are varied and involve multiple systems. Majority of infants are asymptomatic at the time of birth, but if untreated symptoms develop within weeks or months.  The characteristic cutaneous manifestation in CS is erythematous maculo-papular rash followed by scaling with frequent involvement of palms and soles. Similar presentation was described by Kim et al. in a 3-month old infant.  Patients may present with hepatosplenomegaly. In concert with this the index case showed clinical and biochemical evidence of hepatitis. Thrombocytopenia is common in CS, due to platelet trapping in the spleen, was observed in the reported case. Rhinitis and condylomatous lesions over mucus membranes are common.  Painful osteochondritis and periostitis occur frequently in the long bones of the upper and lower limbs. Similar pathological bone changes were noted in the case under study. Moreover, CS can present with only bone involvement and without exhibiting cutaneous manifestations.  Koh et al. in a retrospective review of 13 cases of CS have also observed osteochondritis and periostitis in all the nine patients in whom radiography was carried out.  In a large retrospective review of 302 clinically suspected cases of CS, bone changes were found in 197, which included periostitis (52%), osteitis (15%) and metaphyseal changes (33%) and more than one lesion in 36%.  The common neurological manifestations reported in early CS are postneonatal Erb's palsy, pseudoparalysis and meningitis.  Banapurmath et al. have reported a case of CS presenting with hypotonia associated with brisk reflexes and extensor plantar response. 
The mainstay of prevention of CS is early diagnosis and appropriate treatment of syphilis infected mothers, which can only be achieved by making antenatal care accessible to all pregnant women. Routine antenatal serological test must be carried out early in pregnancy and repeated if necessary in the third trimester. An early negative test may mean either the mother is incubating syphilis or may have acquired it later in pregnancy. If the mother is found to have syphilis, CS will invariably be prevented if treatment is completed at least 2 weeks before delivery. 
| References|| |
Gurlek A, Alaybeyoglu NY, Demir CY, Aydogan H, Coban K, Fariz A, et al.
The continuing scourge of congenital syphilis in 21 st
century: a case report. Int J Pediatr Otorhinolaryngol 2005;69:1117-21.
American Academy of Pediatrics. Report of the Committee on Infectious Diseases. Red Book. 26 th
ed. Elk Grove Village, IL: American Academy of Pediatrics; 2003. p. 595-607.
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.
Feigin RD, Cherry JD. Syphilis. In: Textbook of Pediatric Infectious Diseases. 4 th
ed. Philadelphia: WB Sanders; 1998. p. 1543-56.
World Health Organization. Eliminating congenital syphilis. A global health priority. Geneva: World Health Organization; 2005.
Li Y, Gonik B. Is congenital syphilis really congenital syphilis? Infect Dis Obstet Gynecol 2006;2006:81629.
Kim HY, Kim BJ, Kim JH, Yoo BH. Early congenital syphilis presenting with skin eruption alone: a case report. J Am Med Assoc 2001;286:529-30.
Fan P, Fu M, Liao W, Luan Q, Hu X, Gao T, et al.
Early congenital syphilis presented with exclusive bending pain of extremity: case report. J Dermatol 2007;34:214-6.
Rasool MN, Govender S. The skeletal manifestations of congenital syphilis. A review of 197 cases. J Bone Joint Surg Br 1989;71:752-5.
Ikeda MK, Jenson HB. Evaluation and treatment of congenital syphilis. J Pediatr 1990;117:843-52.
Banapurmath CR, Narasareddy B, Banapurmath SC, Muganagowda, Kesaree N. Unusual presentation in early congenital syphilis. Indian Pediatr 1994;31:843-6.
Schofield CB. Sexually transmitted disease surveillance. Br Med J (Clin Res Ed) 1982;284:825.
[Figure 1], [Figure 2], [Figure 3]