Approximately eight weeks after the initial presentation of a chancre, a generalized infection ensues. This is a result of proliferation and systemic dissemination of T. pallidum and persists until a sufficient immune response develops. Constitutional symptoms including fever, headache, arthralgias, malaise, pharyngitis, and generalized lymphadeno-pathy are common. The dominant feature of secondary syphilis is a mucocutaneous rash, which appears in 90% of patients. While there is significant variation, the characteristic rash begins as small pink to red macules symmetrically distributed on the trunk and extremities, also involving mucous membranes. This will progress to a papular rash and spread to the distal extremities to involve the palms and soles. Eventually the rash will develop a coppery color and become papulosquamous. It will often have the appearance of pityriasis rosea and follow skin cleavage lines, although distinguishable by its involvement with the palms and soles. The timeframe for resolution of this rash is quite variable, ranging from a few days to several months. Sometimes the papules coalesce into moist, broad plaques, called condyloma lata, that are typically gray-white in appearance. These plaques are highly infectious and tend to form in moist skin folds.
With the hematogenous spread of the bacteria, nearly any organ system can be involved in secondary syphilis. The kidneys may demonstrate acute nephrotic syndrome and glomerulonephritis secondary to immune-complex injury. Direct liver invasion will lead to syphilitic hepatitis. The intestine may be involved, showing wall thickening or ulceration, which sometimes can be confused with malignancy. Synovitis and periosteitis have also been described, and uveitis is not uncommon.
One of the areas in which head and neck manifestations become apparent is with central nervous system (CNS) involvement, which may occur in up to 40% of secondary syphilis. This most commonly presents with headache and meningismus; acute aseptic meningitis will be apparent in 1% to 2% of patients (7). Symptoms of visual disturbance, hearing loss, tinnitus, dizziness, and facial weakness may appear with cranial nerve or temporal bone involvement. Nerves II through VIII are affected most frequently. Labyrinthine involvement in secondary syphilis is more likely to produce abrupt, bilateral, rapidly progressive hearing loss, with vestibular symptoms less frequent. CNS involvement may also produce various distal neurologic deficits. The likelihood of progressing to late neurosyphilis is increased with CNS involvement during secondary syphilis; approximately 8% to 10% of untreated patients will do so (7).
Other head and neck manifestations of secondary syphilis are related to the initial mucocutaneous rash (Fig. 3). Facial lesions will follow the hairline of the temporal and frontal scalp and also present with cracking papules at the corners of the lips. Involvement of hair follicles can lead to temporary alopecia in the scalp, beard, eyebrows, and eyelashes. There may be superficial scaling of the skin, including the face and neck, termed "papulosquamous syphilids." Hypopigmented lesions called collaris veneris may appear on the lateral neck. Similar to the condylomata lata that develop in moist skin folds, the mucous membrane lesions of the rash may coalesce into lesions known as mucous patches (Fig. 4). They may present throughout the upper aerodigestive tract. Mucous patches are usually asymptomatic and appear as a slightly elevated flat plaque covered by a silvery gray membrane with a mildly erythematous periphery. As with the condylomata lata, these lesions are teeming with spirochetes and are highly infectious. They may become painful with secondary infection. Laryngeal involvement may produce laryngitis and hoarseness,
FIGURE 3 The rash of secondary syphilis can involve the face, as seen in this image illustrating the annular papulosquamous lesions. Source: Courtesy of the CDC Public Health Image Library; special thanks to Susan Lindsley.
while nasal involvement tends to present as acute rhinitis with scant thick discharge and mucosal inflammation.
Secondary syphilis symptoms persist for a variable time frame, but generally will resolve within one year. Once the lesions of secondary syphilis have resolved, an individual is considered to be in the latent phase. The first year of this period is considered the early latent phase, during which infected individuals maintain a higher potential for disease transmission. Relapses of secondary syphilis do occur and tend to do so in the early latent phase. Immunocompromised patients are at greater risk for recurrence, which tends to be milder than the initial symptoms. If an individual had not been diagnosed in the primary stage, consistent clinical symptoms and positive serologic testing will confirm syphilis in the secondary stage.
Latent syphilis is defined as the period of persistent positive testing with a specific treponemal antibody assay in the absence of disease manifestations. Prospective data regarding untreated syphilis indicate that one-third of patients will clear the disease, one-third will persist with latent syphilis, and one-third will progress to late or tertiary syphilis (8). With the advent of penicillin treatment, late syphilis is rarely seen today. When the disease does advance to the tertiary stage, it involves a slowly progressive inflammatory process. Latent syphilis can persist for years to decades before the manifestations of late syphilis present. As with secondary syphilis, tertiary syphilis can involve nearly any organ
FIGURE 4 A mucous patch characteristic of secondary syphilis. These lesions affecting the mucosal surfaces of the oral cavity and oropharynx are teeming with Treponema pallidum and are highly infectious. Source: Courtesy of the CDC Public Health Image Library; special thanks to Susan Lindsley.
FIGURE 5 When syphilis goes untreated, gum-mas may develop nearly anywhere during the tertiary stage. Though benign, these lesions are locally destructive as demonstrated by this nasal gumma. Source: Courtesy of the CDC Public Health Image Library; special thanks to Susan Lindsley.
system; however, the hallmark findings include gummatous syphilis, neurosyphilis, and cardiovascular syphilis.
The gumma is a granulomatous lesion that can appear in any organ but typically affects the mucocutaneous surfaces and skeletal system. While considered benign, they can be locally destructive, and symptoms, if any, are related to the organ system involved. They vary in size from microscopic nodules to large masses. They begin as irregularly shaped nodules or plaques with a tendency toward central necrosis and ulceration (Fig. 5). When skin lesions ulcerate, they tend to heal with thin, atrophic scars and leave punched-out lesions. Though gummas can develop anywhere, there is a predilection for the arms, back, and face, and may be triggered by minor trauma. Complications within the head and neck can include palatal perforation, saddle-nose deformity secondary to destruction of nasal cartilage, and glossitis. Gummatous involvement of the larynx can produce hoarseness or late complications of subglottic stenosis, vocal cord adhesions, or arytenoid fixation. Gummas can also be seen in the middle ear with resulting tympanic membrane perforation or erosion of the ossicles. Biopsy of a lesion often fails to demonstrate spirochetes and it is thought that gummas represent an intense inflammatory response to a few bacteria. The lesions tend to respond well to antibiotic therapy.
While infrequently seen now, neurosyphilis had significant impact in the past. It is estimated to have caused 5% to 10% of first-time admissions to U.S. mental health hospitals prior to World War II and widespread availability of penicillin (9). As discussed previously, hematogenous dissemination and direct invasion can produce acute neurosyphilis during the secondary stage; however, the CNS can also be involved in tertiary neurosyphilis. Most commonly, this infection is asymptomatic and picked up with an abnormal finding on cerebrospinal fluid (CSF) analysis. This may occur in up to 40% of untreated syphilitics (7). As this asymptomatic infection is curable with antibiotic therapy, lumbar puncture should be performed if the adequacy of prior treatment is in question.
The pathology of symptomatic tertiary neurosyphilis is divided among parenchymatous and meningovascular neurosyphilis, although a combination of the two is nearly always present. The former is an insidious process involving direct destruction of neurons and has a peak incidence of 10 to 20 years after infection. Cerebral cortex destruction can cause general paresis while involvement of the spinal cord may lead to a clinical entity known as tabes dorsalis. This results from demyelinization of the posterior column as well as dorsal roots and ganglia. The manifestations include hypotonia, areflexia, a wide-based ataxic gait, paresthesias, sudden paroxysms of lower limb pains known as lightning pains, bowel and bladder dysfunction, and loss of positional, vibratory, and temperature sensation. Optic involvement is also common, with most cases demonstrating the classic Argyll-Robertson pupil that is unreactive to light but will accommodate to near vision. Gun barrel sight also develops with progressive degeneration of the optic nerve working inward from the periphery, leaving the patient with intact vision but a narrowed visual field. Though rarely seen any longer, tabes dorsalis was the most common presentation of late syphilis prior to penicillin treatment. Parenchymatous disease also produces cognitive decline and psychiatric symptoms with hallucinations and slowly progressive dementia.
Meningovascular neurosyphilis presents slightly earlier, usually 5 to 10 years after infection. As opposed to the destructive nature of parenchymatous disease, this is an inflammatory process and is characterized by endarteritis obliterans involving the small vessels of the meninges, brain, and spinal cord. The symptoms typically begin abruptly relating to multiple small areas of infarction, most commonly in the distribution of the middle cerebral artery. The clinical outcome entails a wide spectrum but can include hemiparesis, aphasia, and seizures.
The aspect of neurosyphilis most relevant to the otolaryngologist is the phenomenon of otosyphilis, historically known as luetic otitis or labyrinthitis. While otic involvement can develop at any stage of the disease, it is typically seen as a late manifestation of tertiary neurosyphilis. The pathology and symptoms seen in late otosyphilis are distinct from what is seen in early disease. While otic involvement in early syphilis is related to treponeme-induced labyrinthitis and direct neuritis of cranial nerve VIII, late otosyphilis demonstrates the obliterative endarteritis characteristic of tertiary neurosyphilis. This produces a periostitis of the otic capsule, particularly the semicircular canals. Gummatous involvement may enhance the osteitis and periostitis. Eventually, the membranous labyrinth undergoes atrophy and fibrosis. The endolymphatic sac and duct become narrowed. Middle-ear involvement can produce fibrosis of the ossicles with a resulting conductive hearing loss (10). The hearing loss of late otosyphilis begins at high frequencies but may progress to complete loss if untreated. While early syphilis usually entails abrupt, bilateral loss, tertiary disease tends to have an indolent nature and often begins quite asymmetrically, so as to seem only a unilateral loss. Vestibular symptoms are much more prevalent in tertiary otosyphilis and often are associated with the tinnitus and fluctuating hearing of Meniere's disease. Tertiary otosyphilis classically presents between 5 and 15 years after infection but may develop as late as 50 years later. Coinfection with HIV tends to accelerate and intensify the process, with symptoms often appearing within the first five years and progressing more rapidly (11).
The final area of particular interest in tertiary syphilis is cardiovascular involvement. Similar to the meningovascular pathology of neurosyphilis, the cardiovascular system is also affected by endarteritis obliterans. It affects the vaso vasorum, predominantly of the ascending aorta, and appears 10 to 30 years after the initial infection. The result is necrosis of the elastic tissue of the medial aortic wall, leading to saccular aneurysms. Affected individuals are often asymptomatic, with aneurysms detected on chest radiography performed for other reasons. Proximal disease may lead to stretching of the aortic valve and insufficiency, which in turn may lead to left ventricular failure. The aneurysms rarely dissect due to extensive scarring but can potentially rupture. Symptomatic disease presents in approximately 10% of untreated syphilitics, but pathologic lesions have been found at autopsy in up to 83% of those with untreated neurosyphilis (12). With regard to otolaryngo-logic manifestations, these aneurysms are of interest due to potential compression against the recurrent laryngeal nerve and resulting dysphonia.
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