Two recent cases of tertiary syphilis

ARTICLE

In spite of the recent increasing incidence of early syphilis in the USA [1-3] and in Germany [4, 5], cases of tertiary syphilis are nowadays rarely seen in developed countries. However some cases are still being reported by some African [6] and Eastern European countries [7].

This is mainly due to higher standards of medical care which lead to more frequent requesting of serological tests and to the widespread use of antibiotherapy for tonsillitis and other common respiratory infections. No other disease has ever been more dramatically affected by antibiotics since the introduction of penicillin in 1944 [1, 8]. In only 3 years the syphilis incidence decreased 18 times, leading the American Board of Dermatology and Syphilology to drop the last word from its name [1].

The reported cases of early syphilis have been kept at stable levels for the last 6 years in Portugal [9]. However we think that the official figures for the prevalence of syphilis in our country, as in other countries, are inaccurate. This is due to a negligent reporting system, as efficient reporting represents a time-consuming task for the physician.

Most of our knowledge of tertiary syphilis is derived from the Oslo study, in which 2,181 early syphilis cases were untreated because it was thought that the side effects of the mercury therapy employed in those days ("two minutes with Venus, two years with Mercury") were more harmful than the progression of the disease itself. In Table 1 we can see the proportion of the various forms of tertiary stages in those cases of untreated syphilis [1].

In 1996 we observed in our Dermatology Department two rare cases of tertiary syphilis, which we are now going to describe.

Case reports

Case 1

A female, 55 years old, divorced, with multiple sexual partners, came to us in January 1996 complaining of pruritic skin lesions, with one year of evolution. These were erythemato-violaceous nodules, coalescing into annular plaques with elevated and well defined borders, psoriasiform scaling and the center tending to resolution. In some lesions we observed noncontractile atrophy. We could see four of these lesions, on the right buttock and scapula and on both thighs (Fig. 1).

For the last ten years she has had action tremor and for the last year she has complained of paresthesias on the hands and feet. She reported the appearance of two asymptomatic alopecic patches, 2 cm in diameter, two years before, with self-resolution in about two months.

On the inguinal regions there were several rubbery painless nodules with diameters between 1 and 2 cm, compatible with lymphoadenopathies and confirmed by computerized axial tomography (CAT) scan of the area. The remaining physical examination was normal, namely neurological and ears, nose and throat (ENT) examinations.

She had normal blood tests, except for an elevated erythrocyte sedimentation rate (27 mm, 1st hour). Chest X-ray, viral serologies (for hepatitis B/C and HIV 1/2), immunological study and tuberculin skin test were all negative or normal.

The specific treponemic tests were always reactive. In January 1996 the serum reagins were negative becoming thereafter positive (Table II).

Biopsy of a lesion revealed a dense lympho-histiocytic infiltrate with some plasma cells. The capillaries showed endothelial proliferation and swelling. Granulomas were absent on the skin fragment observed and the PAS and silver stains were negative.

The investigation for cardiovascular involvement, with echocardiography and chest X-ray, was negative, as well as the cerebral CAT scan and the biochemical, cytological and serological (VDRL, FTA-ABS and TPHA) analysis of the cerebrospinal fluid (CSF).

With the diagnosis of benign tertiary syphilis she was treated with 3 doses of 2.4 million U benzathine penicillin G intramuscular injections, one per week. She showed a marked clinical improvement, now only exhibiting residual hyperpigmentation, with slight atrophy at one of the lesions.

The patient is still on observation for repeated nontreponemal serological tests, which so far have revealed a satisfactory evolution.

Case 2

A female, 33 years old, single, mentally handicapped, from a low socio-economic class and with promiscuous sexual habits.

She was referred to us in April 1996 for cutaneous lesions, with two weeks of evolution, according to the patient. On inspection, she presented erythematous plaques, with psoriasiform scaling, well defined borders, sometimes annular in configuration, with the center tending to noncontractile atrophy. In some of these lesions we could see round ulcers with well defined, elevated borders and crusted surfaces. We observed these lesions on the face (supraciliar and interciliar regions, nose and upper lip) and on the trunk (Figs. 2 and 3). There were no adenopathies and the neurological and ENT examinations were also normal.

In the blood tests we detected an elevated erythrocyte sedimentation rate (94 mm in the 1st hour) and high levels of gamma-glutamiltransferase (73 U/l. Normal: 7-49 U/l). The chest X-ray, viral serologies (for hepatitis B/C and HIV 1/2), immunological study and tuberculin skin test were all negative or normal. The specific treponemic tests, FTA-ABS and TPHA, were reactive, but the VDRL on the serum was negative on two repeated measurements. Biopsy of a skin lesion revealed a dense plasmocytic infiltrate with discrete endothelial swelling, but without granulomas. The PAS and silver stains were negative.

The investigation for cardiovascular involvement, with echocardiography and chest X-ray, was negative. The cerebral CAT scan showed a moderate cortical atrophy and the biochemical, cytological and serological (VDRL, FTA-ABS, TPHA) analysis of the CSF were negative.

With the diagnosis of benign tertiary syphilis she was treated with 3 doses of 2.4 million U benzathine penicillin G intramuscular injections, one per week. In a few weeks we could only observe atrophic scars in the site of the lesions.

The patient is still on observation for repeated nontreponemal serological tests, which so far have also revealed a satisfactory evolution.

Discussion

The rare recent reports of tertiary syphilis in the literature concerned mainly African patients [6]. There is also a report concerning a French female, who used to be treated by homeopathic medicine only, avoiding thus penicillinotherapy [10], and an American woman with a long-standing involvement of the face, misdiagnosed as discoid lupus erythematous for 30 years [11].

The two cases that we have just presented are thus a rarity in the European Union. This picture is quite different from that seen when Sir William Osler stated that "Syphilis simulates every other disease. It is the only disease necessary to know. One then becomes an expert dermatologist (...)" [1].

The initial negativity of the VDRL test added some difficulties to both diagnoses. However it is well known that the course of the titers of VDRL in a non-treated late syphilis, with a low treponemic charge, can oscillate between detectable and non-detectable levels, being negative in 25% of the patients [12, 13].

These cases stress the necessity of a high degree of clinical suspicion when reaching a diagnosis of tertiary syphilis. It is important to obtain the serologies and a histopathological examination of the skin lesions, keeping in mind that even these can be misleading. Thus we should not be afraid of performing a therapeutic challenge with benzathine penicillin, after ruling out neurosyphilis with biochemical, cytological and serological CSF examinations.

Tertiary syphilis, latent syphilis of indeterminate or more than 1 year's evolution and syphilis in an HIV⁺ patient should undergo a complete investigation to rule out systemic involvement, mainly neurosyphilis. The importance of these reports is thus to stress the importance of keeping alive the physician's knowledge of syphilis, as its tertiary stages could easily be the cause of embarrassing misdiagnosis, leading to the patient being treated for the wrong disease, sometimes for decades [11].

REFERENCES

1. Sanchez M, Luger AFH. Syphilis. In: Fitzpatrick TB, Dermatology in General Medicine. New York: McGraw-Hill Inc., 1993: 2703-43.

2. McArthur JC, Harrison MJG. Cerebral infections in AIDS: Neurosyphilis. Infect Med 1997; 14: 65-74.

3. Hook III EW, Marra CM. Acquired syphilis in adults. N Engl J Med 1992; 326: 1060-9.

4. Haustein EU. Syphilis incidence in West Saxony (Germany) between 1983 and 1992. J Eur Acad Dermatol Venereol 1995; 4: 97-9.

5. Petzoldt D. Anstieg der Syphilis in der Alpen-, Donau-, Moldauregion. Hautarzt 1996; 47: 335.

6. Sekkat A, Sedrati O, Derdabi D. La syphilis tertiaire cutanéo-muqueuse. Ann Dermatol Venereol 1994; 121: 146-51.

7. Anton'ev AA, Shaparenko MV, Toporovskii LM, Zabanova EV. Tretichnyi aktivnyi sifilis u bol'nogo posle trt'ego zarazheniia (Tertiary active syphilis in a patient after a third infection). Vestn Dermatol Venereol 1990; 12: 40-3 (Medline^® abstract).

8. Morton RS. The treponematoses. In: Champion RH, ed. Textbook of Dermatology. London: Blackwell Scientific Publications, 1992: 1085-115.

9. Direcção-Geral da Saúde, Doenças de Declaração Obrigatória 1991-1995 (Estatísticas), Lisboa, 1997; 104 p.

10. Drobacheff C, Moulin T, van Landuyt H, Merle C, Vigan M, Laurent M. Syphilis cutanée tertiaire avec symptômes neurologiques. Ann Dermatol Venereol 1994; 121: 34-6.

11. Chung G, Kantor GR, Whipple S. Tertiary syphilis of the face. J Am Acad Dermatol 1991; 24: 832-5.

12. Esteves JA, et al. Sífilis. In: Dermatologia. Fund. Calouste Gulbenkian, Lisboa, 1992: 1322.

13. Rosen T, Brown TJ. Cutaneous manifestations of sexually transmitted diseases. Med Clin North Am 1998; 82: 1081-104.