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].
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