Syphilitic anterior uveitis (see Fig. 12.1) is granulomatous in two thirds of patients [2] and bilateral in half. Interstitial keratitis, iris nodules, dilated iris vessels, and iris atrophy may also be seen. The most common form of posterior uveitis is multifocal chorioretinitis, but other manifestations include focal chorioretinitis (see Fig. 12.2), pseudoretinitis pigmentosa, retinal necrosis, neuroretinitis, optic neuritis (see Fig. 12.3), and acute zonal occult outer retinopathy. A pale optic nerve head from prior syphilitic optic neuritis may mimic glaucomatous optic atrophy. Chorioretinitis was the type of uveitis seen in 15 of 20 patients with syphilitic posterior uveitis in one review [1]. A specific type of focal chorioretinitis, acute posterior placoid chorioretinitis, has been described in syphilis and is characterized by large, often solitary yellow lesions that are typically in the macula [3]. Retinal vasculitis may occur in ocular syphilis, and branch retinal vein occlusions have been described [4].

Uveitis may occur in either congenital or acquired syphilis. Typical ocular findings in congenital disease include interstitial keratitis and so-called salt–and–pepper fundi. Interstitial keratitis does not usually occur until the patient is a teenager or young adult. It may be accompanied by anterior uveitis. The patient may have no other stigmata of congenital syphilis, but other possible features include prematurity, low birth weight, nonimmune hydrops fetalis, placental and umbilical cord abnormalities, fever, hepatomegaly, failure to thrive, rhinitis, maculopapular rash, vesicular rash (pemphigus syphiliticus), condyloma lata, jaundice, and hematologic, musculoskeletal, neurologic, pulmonary, and/or renal disease.

In acquired syphilis, uveitis may occur in secondary or tertiary syphilis. The chancre of primary syphilis, a painless lesion that develops at the inoculation site (usually genital area) an average of 3 weeks after inoculation, may have been unnoticed by the patient. The chancre lasts 3–6 weeks then spontaneously resolves. Secondary syphilis typically begins 2–8 weeks after the chancre, but this period is variable. With ocular manifestations that occur during secondary syphilis, eye symptoms are often acute. In older reports, the most common ocular finding in secondary syphilis was iritis, which accounted for more than 70 % of eye findings [4]. More recent reports suggest that posterior segment inflammation predominates; these reports include larger numbers of patients with concomitant HIV infection and low CD4 cell counts, which may predispose to more aggressive and posteriorly-located disease [5, 6]. Other manifestations of secondary syphilis may include fever, rash (classically involving the palms and soles—see Fig. 12.4), swollen lymph glands, sore throat, patchy hair loss, headaches, weight loss, muscle aches, and fatigue.

In contrast, when ocular syphilis develops in tertiary disease, patients often have slowly progressive decrease in vision as their only symptom. The eye findings are protean and include all of the above-listed findings. In contrast to patients with secondary disease, patients with tertiary disease often are middle-aged or older. They often have no knowledge of prior exposure to syphilis, which likely occurred decades earlier. The diagnosis may be missed if only nontreponemal tests are checked, because these tests are often negative in tertiary syphilis. In a series of 50 patients with a reactive treponemal test [absorbed fluorescent treponemal antibody (FTA-ABS)] and eye findings consistent with active or inactive ocular syphilis (e.g., chorioretinitis, optic atrophy, iritis, interstitial keratitis), the average age was 59, and the VDRL was reactive in only 24 % [7].