Syphilis presenting as acute multifocal retino-choroiditis

We describe a patient who presented with an unusual bilateral migrating luetic chorioretinitis.

CASE HISTORY

A 44-year-old celibate Caucasian man presented with a 1-day history of decreased left vision. There was no significant past history. Visual acuities were 6/6 in the right eye and 6/36 in the left. There were cells (+) in the left vitreous and an area of chorio-retinitis below the left macula. A fluorescein angiogram showed associated extensive leakage. Routine blood tests, including full blood count and C-reactive proteins, were normal. The chest X-ray was unremarkable. His serum rheumatoid factor was 260 IU/mL (N=<20); autoantibody screening was negative; and the serum angiotensin converting enzyme was raised at 62 (N=8-52). A diagnosis of idiopathic inflammatory chorioretinitis was made.

On commencing oral prednisolone 60mg/day the chorioretinitis subsided and visual acuity improved to 6/6. The systemic steroids were reduced and 2 weeks later vision in the right eye deteriorated to 6/24. Examination showed mild vitritis and the fluorescein angiogram indicated late leakage at the right posterior pole. Intravenous methylprednisolone 1g/day was commenced for 3 days, and the visual acuity in the right eye improved to 6/9. He was maintained on oral steroids (20 mg/day) and cyclosporin (150 mg/day); but further visual deterioration occurred over the next 10 days to hand movements in both eyes. He exhibited a migrating multifocal retinitochoroiditis with whitish placoid retinal lesions (Figure 1 [top] and Figure 2). The peripheral phlebitis and mild disc oedema were noted bilaterally.

Figure 1.

A fundus picture (both eyes). Top: showing multifocal placoid retino-choroiditis. Bottom: 4 weeks after commencing penicillin showing pigmentary changes at the maculae [in colour online]

Figure 2.

A fluorescein angiogram of the left eye showing leakage. Early and late phases are indicated by numbers: 1 being the earliest phase and 4 being the last

Serology excluded toxoplamosis, Lyme disease and Weil's disease. A polymerase chain reaction was done on the cerebrospinal fluid to exclude herpes simplex, varicella zoster and cytomegalovirus. An echoencephalogram was normal.

Three months after initial presentation he developed a generalized maculo-papular rash with involvement of the palms, soles, lymphadenopathy and oral ulcerations. There was no evidence of genital ulceration. Although not deemed at risk of sexually transmitted infection, syphilis serology was also undertaken. Treponemal IgG and triethylene thiophosphoramide were positive with a VDRL of 1:64. Lumbar puncture found cerebrospinal fluid lymphocytosis, raised proteins and a positive Treponemal IgG.

He was admitted for a 17-day course of intravenous benzyl penicillin for the treatment of secondary neurosyphilis. A full sexual history was taken at this stage, and it was found that 3 months earlier he had been sexually assaulted by two men, which had involved unprotected receptive oral and anal intercourse. There had been no other sexual encounters with men or women for several years prior. Genitourinary screen and HIV testing were negative. (The UK national guidelines on the management of syphilis are available at [http://www.bashh.org].

Within a week his retinal oedema subsided. Within 3 weeks his retinal lesions had disappeared. His visual acuity improved to 6/9 bilaterally. At last review mild residual pigmentary changes at both fovea persist (Figure 1 [bottom]). Follow-up syphilis serology at 3, 6 and 12 months showed a gradual drop in VDRL titres becoming serofast at a level of 1:8.

DISCUSSION

Our patient was a Caucasian man, who had been celibate for several years, presenting with acute multifocal migratory retino-choroiditis secondary to syphilis.

Syphilis had been recognized as a common cause of intraocular inflammation during the early half of the 20th century, but the occurrence declined dramatically with the advent of penicillin. The past decade has seen the reemergence of infectious syphilis in the UK.^1-3 Primary syphilis is characterized by a chancre and lymphadenopathy; secondary syphilis is a systemic illness with a characteristic maculopapular rash and a wide range of systemic involvement including ocular disease. The most common ocular manifestation is anterior uveitis, though posterior, intermediate and panuveitis have also been described. Optic neuritis, scleritis and interstitial keratitis are less common.⁴

Diagnosis of syphilis was delayed in our patient for several reasons. Chorioretinitis as the initial presentation though described is rare.⁵ The clinical course of the disease was no doubt confounded by the use of immunosuppressants, and the deemed irrelevance of sexual history taking to the presenting complaint.

Although the majority of recent syphilis cases have been diagnosed at genitourinary clinics in the UK,² it seems likely that the cases of secondary syphilis, especially if associated with less of the usual symptoms, may present to other specialities including eye departments.⁶

Early screening for syphilis should be routine for any unusual cause of uveitis, however improbable the diagnosis may seem. A missed diagnosis can lead to devastating ocular and systemic consequences.