Educational materials are taken from

A.Zagoroulko, T.Filonenko. Digest on

pathomorphology. – Simferopol, 2007.

SYPHILIS
Syphilis (lues) is a sexually transmitted disease of mankind caused by the spirochete
Treponema pallidum.
Stages of syphilis:
1. Primary (the chancre).
2. Secondary (disseminated).
3. Tertiary (with lesions of deep organs following a latent period of 2 to 20 years or more).
The chancre develops at the site of inoculation in 10 to 90 days (average 21 days) and has
a characteristic “luetic vasculitis”, in which endothelial cells proliferate and swell, and the walls
of the vessels become thickened by lymphocytes and fibrous tissue.
Morphology
In primary Syphilis, the chancre is a slightly elevated, firm, reddened papule, up to
several centimeters in diameter that erodes to create a clean-based, shallow ulcer. Histologically,
the chancre contains an intense infiltrate of plasma cells, with scattered macrophages and
lymphocytes and an obliterative endarteritis. The regional nodes are usually enlarged and may
show nonspecific acute or chronic lymphadenitis, plasma cell-rich infiltrates, or focal epithelioid
granulomas. The combination of chancre, lymphangitis, and lymphadenitis is called primary
syphilitic complex.
Secondary Syphilis. It presents as a widespread skin rash (pox) of varying appearance,
ulceration of mucous membranes, generalized lymphadenopathy, damage to various individual
organs and tissues. There are constitutional effects – particularly fiver and anemia. The essential
pathology is the presence of very numerous spirochaetes accompanied by focal infiltration of
lymphocytes, plasma cells and macrophages with mild arteritis. Infectivity is very high. Tissue
destruction is minimal and healing occurs without scarring. A latent stage of long duration is
followed in 35% of cases by tertiary syphilis.
Tertiary (Late) Syphilis. The lesions, which may occur at any time for many years after
healing of the secondary phase, offer striking contrasts. This stage is characterized mainly by
local destructive lesions, the result of cell-mediated immune reactions (T-cells) causing necrosis
of tissue. It occurs years after the initial infection and most frequently involves the aorta, the
central nervous system, and the liver, bones and testes (gummas).
The main forms are:
1. Gumma. This is a localized area of necrosis, which may affect large parts of any organ
or tissue but particularly bones, testis and liver and looks like white-gray and rubbery
formation. In the liver, gumma may produce the coarsely nodular pattern of cirrhosis,
termed hepar lobatum because of the simulation by the deep scars of multiple lobes. Bone
and joint gummas lead to areas of cortical and articular destruction. Pathologic features
and joint immobilization may result. Testicular gummas often cause painless enlargement
of the affected testis, thus simulating a tumor.
Histologically, the gummas contain a center of coagulated, necrotic material and margins
composed of plump or palisaded macrophages and fibroblasts surrounded by large
numbers of mononuclear leukocytes, chiefly plasma cells.
2. Syphilitic aortitis. The aorta is affected by an infiltration of lymphocytes and plasma
cells beginning around the vasa vasorum and extending into the media, causing
weakening due to focal destruction (windowing) of the specialized elastic tissues. There
is compensatory irregular thickening of the intima (tree-bark appearance), but the
important effect is expanding aneurysm formation.
 3. Neurological Syphilis. Neurosyphilis takes one of several forms, designated
meningovascular Syphilis, tabes dorsalis, and general paresis.
Meningovascular – mainly affects the meningeal blood vessels and causes neurological
impairment secondary
Parenchymatous:
a) General paralysis of the insane – severe destruction of cerebral tissue, atrophy of
convolutions, enlargement of ventricales;
b) Tabes dorsales – the damage specifically affected the posterior roots and columns of spinal
cord – is associated with characteristic clinical symptoms due to lose of proprioceptive sensation
in the legs.
Congential syphilis is most severe when the mother’s infection is recent. In perinatal and
infantile Syphilis, a diffuse rash develops. Syphilitic osteochondritis and periostitis affect all
bones. Destruction of the vomer causes collapse of the bridge of the nose and, later on, the
characteristic saddle nose deformity. Periostitis of the tibia leads to excessive new bone growth
on the anterior surfaces and anterior bowing, or saber shin. The liver is often affected severely in
congenital syphilis. Diffuse fibrosis permeates lobules to isolate hepatic cells into small nests.
Gummas are occasionally found in the liver, even in early cases. The lungs may be affected by a
diffuse interstitial fibrosis.
The late-occurring form of congenital syphilis is distinctive for the triad of interstitial
keratitis, Hutchinson's teeth, and eight-nerve deafness.