Oral Manifestations of Tertiary Syphilis: a case report Titinchi F, Morkel JA Department of Maxillo-Facial and Oral Surgery Faculty of Dentistry, University of the Western Cape, Tygerberg, South Africa Introduction Syphilis is caused by the anaerobic filamentous spirochete, Treponema pallidum. In the past decade there has been a significant rise in the prevalence of infective syphilis in developing countries. The changing epidemiology of syphilis reflects the rise in HIV infection, falling use of barrier methods of contraception, high numbers of sexual partners, lack of relevant knowledge, etc. Syphilis can be classified as either congenital or acquired. The acquired form can be classified as primary, secondary, latent, and tertiary, depending on the elapsed time after exposure. All stages may present with oral lesions. Table 1: Summary of oral-facial manifestations of Syphilis Stage of Syphilis Oral-facial features Primary - Chancre - Non-tender cervical lymphadenopathy Secondary - Mucous patch - Maculopapular eruptions - Syphilitic leukoedema Tertiary - Gumma lesions in palate and/or tongue - Atrophic and interstitial glossitis - Syphilitic leukoplakia - Syphilitic sialadenitis - Trigeminal neuropathy Congenital - Hutchinson’s incisors/Mulberry molars - Facial deformity - High arched or gothic palate - Maxillary hypoplasia “Bulldog” jaw - Saddle shaped deformity of nose - Frontal bossing Fig. 1: Intraoral view showing ulcerative lesions in hard palate. Fig. 2: Intraoral view of the acrylic obturator in place. Discussion Tertiary syphilis can be defined as appearance of new lesions in untreated patient after one year of primary lesions. The typical notorious lesion of this stage is gumma. It shows chronic hypersensitivity reaction against T pallidum. In the oral mucosa, gumma usually affects hard palate and start with a well defined central ulcerative lesion. Gradually it increases in size and perforate in the centre to the nasal cavity. There are chances of malignant changes in gummatous lesion so regular biopsy is recommended in every six months. Gumma is often asymptomatic but very destructive lesion at this stage and if not manage on time; it can causes perforation of underlying structure and cause permanent deformity. Systemic complications can seriously affect the cardiovascular and nervous systems but are very uncommonly seen. Management of tertiary syphilis is summarized in Table 2. Penicillin is the main treatment modality while obturators are a successful method of managing speech and masticatory problems. Surgery is another option but extensive scarring in syphilitic lesions makes any attempt at palatal repair hazardous. Ischaemia and necrosis render the tissues more likely to breakdown following surgical repair. The better option seems to be the non-surgical conservative approach. Table 2: Summary of management of oral tertiary syphilis. Oral manifestations of syphilis are not common and palatal perforation related to tertiary syphilis are not usually considered as part of differential diagnosis. Recent studies have focused on the altered presentation in HIV positive men who are at greater risk of tertiary syphilis and have thus increased the likelihood of clinicians witnessing its signs and symptom. Here we present a case of palatal perforation caused by tertiary syphilis to highlight the re-emergence of syphilis as a possible cause of palatal perforation and to keep this as differential diagnosis in mind Case Report A 35 year old male presented at the Department of Maxillo-Facial and Oral Surgery, Tygerberg Hospital in 2014 with a main complaint of an ulcerative lesion on the hard palate that has been present for about four weeks. The patient reported that the lesion is very painful and he was having difficulty swallowing and speaking. Medical history indicated the patient is HIV positive and on anti-retroviral treatment. The patient was also diagnosed with Tuberculosis and was on treatment. Upon extra-oral examination, a discoloured nodule on the tip of nose was present for few years. Bilateral submandibular lymphadenopathy was noted. Intra-orally, a large well-defined 2x2cm punched out ulcerative lesion was noted and involved the roof of the hard palate. On haematological study red cell count, haemoglobin and platelet count were low while erythrocyte sedimentation rate was high. Beside this all routine haematological parameters were within normal limit. The absolute CD4 count was 12 cells/uL Initially a brush biopsy was performed of the intra-oral lesion on the hard palate and results came back as a fungal infection. The patient was placed on Nystatin oral suspension 500000 units PO 6 hourly and Chlorhexidine 0.2% mouthwash. Further blood analysis were conducted as syphilis was suspected. Both T pallidum antibodies and Rapid Plasma Reagin were reactive while bacterial culture was negative for actinomycosis The patient was medically managed on penicillin G. The gumma lesion of the palate became necrotic and healed leaving a large oronasal fistula (Figure 1). The fistula was obturated using an acrylic removable obturator (Figure 2). Initial phase - Antibiotics (Penicillin G, Tetracyclines, etc) - Preventative advice Conservative/Non surgical phase - Palatal obturator/dentures - Speech therapy - Avoid surgery due to risk of further necrosis Surgical/Reconstructive phase - Last resort to close large defects - Standard cleft palate techniques - Local palatal flaps for small defects - Tongue flap (high vascularity) - Microsurgical forearm flaps Conclusion Tertiary syphilis presents with destructive gamma lesions that can cause severe destruction to the orofacial regions. It is important to diagnose these lesions early with the aid of clinical and laboratory investigations. Management is aimed at eliminating the bacterial organisms with antibiotics and to manage the patient conservatively. Stage of Syphilis Oral-facial features Primary - Chancre - Non-tender cervical lymphadenopathy Secondary - Mucous patch - Maculopapular eruptions - Syphilitic leukoedema Tertiary - Gumma lesions in palate and/or tongue - Atrophic and interstitial glossitis - Syphilitic leukoplakia - Syphilitic sialadenitis - Trigeminal neuropathy Congenital - Hutchinson’s incisors/Mulberry molars - Facial deformity - High arched or gothic palate - Maxillary hypoplasia “Bulldog” jaw - Saddle shaped deformity of nose - Frontal bossing Stage of Syphilis Oral-facial features Primary - Chancre - Non-tender cervical lymphadenopathy Secondary - Mucous patch - Maculopapular eruptions - Syphilitic leukoedema Tertiary - Gumma lesions in palate and/or tongue - Atrophic and interstitial glossitis - Syphilitic leukoplakia - Syphilitic sialadenitis - Trigeminal neuropathy Congenital - Hutchinson’s incisors/Mulberry molars - Facial deformity - High arched or gothic palate - Maxillary hypoplasia “Bulldog” jaw - Saddle shaped deformity of nose - Frontal bossing Stage of Syphilis Oral-facial features Primary - Chancre - Non-tender cervical lymphadenopathy Secondary - Mucous patch - Maculopapular eruptions - Syphilitic leukoedema Tertiary - Gumma lesions in palate and/or tongue - Atrophic and interstitial glossitis - Syphilitic leukoplakia - Syphilitic sialadenitis - Trigeminal neuropathy Congenital - Hutchinson’s incisors/Mulberry molars - Facial deformity - High arched or gothic palate - Maxillary hypoplasia “Bulldog” jaw - Saddle shaped deformity of nose - Frontal bossing References 1. 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