Natural course of untreated syphilis – showing time frames and the approximate proportion of persons developing different forms of clinical disease (in parentheses). It is not known what proportion of initially infected persons develop primary or secondary manifestations.
Sutton, M, Dorell, C, Glob. libr. women's med., 2009.
The common manifestations of secondary syphilis are: (i) a generalised skin rash (75–100%) (ii) lymphadenopathy (50–80%) and (iii) mucocutaneous lesions like mucous patches and condyloma lata (40–50%).
Generalised rash, year 1948 (left). Rash involving the palms (and soles) (center). Peri-anal condylomata, year 1944 (right).Example: Condyloma Lata
The patient was a 40 year old woman, with a history of general debility, and of rectal obstruction and discharge. On examination she had numerous warty growths around the vulva and anus. She died of a treatment complications. Her autopsy revealed a massive, chronic inflammatory lesion in the pelvis with a chronic abscess which was draining into the rectum.
The specimen shows the vulva, and around most of the periphery there are flat, warty, thickened areas in the skin. Posteriorly these have become more heaped-up and almost pedunculated. The appearance is that of condylomata lata of syphilis.
Ref: G1-i43-2559 Year 1930
Latent syphilis is the stage of disease during which there are no clinical signs but specific treponemal antibody test are positive.
Tertiary or late syphilis is a slowly progressive inflammatory disease that can affect any organ and have protean manifestations 3 to 30+ years after the initial infection. It can be divided into late "benign" syphilis (gummata), cardiovascular syphilis and neurosyphilis. Syphilitic osteitis is also a feature of late disease.
The gumma is an indolent non-specific granulomatous lesion most commonly occurring in bones and joints, skin and and subcutaneous tissues, but they can occur in any organ. They can vary from microscopic to large masses, from superficial nodules to deep ulcerating lesions, with the general result of local destruction. Involution is followed by scarring.
Example: Gummata of the liverThe patient, a 20 year old woman, was admitted with discharging ulcers on the back of her neck and a productive cough. On examination, there were signs of consolidation at her right lung apex. Her clinical course was steadily downhill to death.
At autopsy, there were gummatous lesions of the back of her neck, the bridge of her nose, and her lung - the latter communicating with a bronchus, and accompanied by an empyema on the same side. Her liver contained a circumscribed but irregular bright yellow lesion, to a certain extent broken up by a dense layer of white, translucent fibrous tissue. The appearance is typical of a gumma.
Ref:D9-i44-1574 Year 1929
Example: Hepar Lobatum
The patient was a 30 year old woman whose Wasserman reaction was strongly positive. Her further course to demise in hospital is not known.
Her liver is markedly nodular and irregular, with deep lines of scarring due to contraction of fibrous tissue and nodules of regeneration in between, the left lobe being more affected than the right. The appearance is typical of hepar lobatum, and is due to healing of multiple gummatous lesions.
Ref: D9-g78-1683 Year 1940
Example: Syphilis of the trachea
The patient, a 26 year old woman, had suffered dysphagia, cough and hoarseness for 6 months before admission and from dyspnoea for 1 week. She died of pulmonary complications.
The specimen consists of both lungs and the trachea. There is marked thickening, irregularity and ulceration of the trachea just above and below the bifurcation. The lungs have a mottled, irregular appearance due mainly to inhaled blood, but there are also areas of collapse and compensatory emphysema. In the upper lobe of the left lung is a partially healed, primary tuberculous focus.
The macroscopic appearance of the trachea suggests carcinoma, but the microscopic picture is that of syphilis.
Ref:R2-i44-0621 Year 1937
Image credit: Yale Rosen https://www.flickr.com/photos/pulmonary_pathology/9727667274/
Glossitis can be a feature of tertiary syphilis and it predisposes to carcinoma, especially in smokers.
Image credit: Morris M. A case of late syphilitic glossitis treated by Salversan (Erlich-Hata). Br Med J. Mar 30, 1912; 1(2674): 712–712.1.
Example: Syphilitic glossitis with superimposed carcinoma
The tongue has been amputated through the posterior third. It shows leukoplakia and on the right is a large, irregular ulcer with indurated edges - the appearance is highly suggestive of a "malignant ulcer".
Microscopy confirmed a squamous carcinoma, which had also infiltrated the cervical lymph nodes.
Ref: D1-n83-2198 Year 1921
SYPHILITIC AORTITIS: The ascending aorta and aortic arch are most often affected. A syphilitic endarteritisinvolving the vasa vasorum in the adventitia results in ischaemic destruction of elastic and muscle fibres within the media. Reparative scarring and vascularisation weaken the wall, leading to dilatation (either diffuse or aneurysmal). The intima undergoes compensatory thickening, on top of which atheroma is then frequently superimposed. Secondary effects of syphilitic aortitis include:
(i) dilatation of the aortic valve ring leading to aortic incompetence, which in turn leads to dilatation and hypertrophy of the left ventricle.
(ii) involvement of the ostia of the coronary arteries, producing cardiac ischaemia.
SYPHILITIC AORTIC VALVULITIS: This is an extension of syphilitic aortitis to the valve cusps, and is thus confined to the aortic valve. There is often a tendency for the free edge of the cusp to be most affected.
Example: Syphilitic aortitis
The patient was a 55 year old white man who had contracted syphilis 25 years previously.
The ascending aorta shows moderate diffuse dilatation and the intima is thickened and wrinkled. Whilst there is no definite thickening of the aortic valve cusps, stretching of the valve ring has led to incompetence and consequent dilatation and hypertrophy of the left ventricle.
Ref: C1-i43-0803 year 1927
Acute syphilitic meningitis is not uncommon during the secondary stage of syphilis. If untreated, up to 10% of patient progress to late neurosyphilis:
- Meningovascular syphilis presents with stroke, usually within 5 years of infection
- General paresis (cortical) and tabes dorsalis (spinal cord) are the main forms of parenchymatous neurosyphilis, presenting 10-20 and 15-20 years after infection, respectively. General paresis is a dementia due to diffuse parenchymal atrophy resulting from chronic meningoencephalitis. Tabes dorsalis is a myelopathy due to chronic inflammatory disease of the dorsal roots and ganglia with associated degeneration of the posterior columns.
- The eyes and ears can be affected by neurosyphilis through optic neuritis/atrophy and VIIIth nerve involvement.
Image credit: http://www.urmc.rochester.edu/libraries/courses/neuroslides/lab3b/slide120.cfm