Tertiary syphilis

Tertiary syphilis usually occurs 1–10 years after the initial infection, however in some cases it can take up to 50 years. This stage is characterized by the formation of gummas, which are soft, tumor-like balls of inflammation known as granulomas. The granulomas are chronic and represent an inability of the immune system to completely clear the organism. They may appear almost anywhere in the body including in the skeleton. The gummas produce a chronic inflammatory state in the body with mass effects upon the local anatomy. Other characteristics of untreated tertiary syphilis include neuropathic joint disease, which is a degeneration of joint surfaces resulting from loss of sensation and fine position sense (proprioception). The more severe manifestations include neurosyphilis and cardiovascular syphilis. In a study of untreated syphilis, 10% of patients developed cardiovascular syphilis, 16% had gumma formation and 7% had neurosyphilis.Neurological complications at this stage can be diverse. In some patients manifestations include generalized paresis of the insane, which results in personality changes, changes in emotional affect, hyperactive reflexes and Argyll-Robertson pupil. This is a diagnostic sign in which the small and irregular pupils constrict in response to focusing the eyes, but not to light. Tabes dorsalis, also known as locomotor ataxia, a disorder of the spinal cord, often results in a characteristic shuffling gait. See below for more information about neurosyphilis.Cardiovascular complications include syphilitic aortitis, aortic aneurysm, aneurysm of sinus of Valsalva and aortic regurgitation. Syphilis infects the ascending aorta causing aortic dilation and aortic regurgitation. This can be heard with a stethoscope as a heart murmur. Contraction of the tunica intima leads to a tree bark appearance that is wrinkly. The aortic valve dilation and subsequent insufficiency leads to diastolic regurgitation and causes massive hypertrophy of the left ventricle. The heart grows so large (over 1,000 grams) that the heart is termed cor bovinum (cow's heart). The course can be insidious and heart failure may be the presenting sign after years of disease. The infection can also occur in the coronary arteries and cause narrowing of the vessels. Syphilitic aortitis can cause de Musset's sign, a characteristic bobbing of the head in synchrony with the heartbeat. The clinical course of these cardiovascular effects causes mediastinal encroachment and secondary respiratory difficulties (dyspnea), difficulty swallowing (dyphagia) and persistent cough because of pressure on the recurrent laryngeal nerve triggering the cough reflex. Pain can stem from erosion of the ribs or vertebrae. Also, the cor bovinum can lead to coronary ostia obstruction and ischemia. The aneurysm developed during the disease course may also rupture, leading to massive intrathoracic hemorrhage and likely death; although the most likely cause of death is the heart failure resulting from aortic regurgitation.

Model of the head of a patient with tertiary syphilis.