Syphilis Presenting as Retinal Detachment and Orchitis in a Young Man With HIV : Sexually Transmitted Diseases

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Case Report

Syphilis Presenting as Retinal Detachment and Orchitis in a Young Man With HIV

Yogo, Norihiro MD; Nichol, Aran Cunningham MD; Campbell, Thomas B. MD; Erlandson, Kristine M. MD

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doi: 10.1097/OLQ.0000000000000078
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CASE REPORT

A 28-year-old previously healthy bisexual man presented to his primary care physician in January of 2012 with complaints of floaters in his right eye. He was referred to an ophthalmologist who made the diagnosis of retinal detachment. The patient underwent vitrectomy of the right eye with intraocular gas tamponade with eventual improvement of his vision. In March of 2012, the patient represented with complaints of floaters in the contralateral left eye and was found to have a large retinal detachment, again requiring vitrectomy with intraocular gas tamponade.

In subsequent follow-up through June of 2012, the patient was noted to have persistent inflammation of the posterior chamber prompting evaluation with a treponemal antibody absorption (FTA-abs), rapid plasma regain (RPR), and human immunodeficiency virus 1 (HIV) enzyme-linked immunosorbent assay. The FTA-abs returned positive with a negative RPR result. A positive HIV enzyme-linked immunosorbent assay was confirmed by Western blot. The patient reported that these test results were negative in March of 2011.

In July 2012, owing to concerns of neuro-ocular syphilis and a prior anaphylactic reaction to penicillin, the patient was admitted for further diagnostic evaluation and penicillin desensitization. Examination revealed left retinal scarring with cataract but no signs of active infection or inflammation. Plasma HIV-1 RNA was 6.24 log10 copies/mL, and CD4 lymphocyte count was 490 cells/µL. Repeat FTA-abs was positive, and the RPR titer was greater than 1:256, indicative of a previous false negative due to a prozone phenomenon. Cerebral spinal fluid (CSF) was notable for 339 white blood cells/µL with 77% lymphocytes, glucose of 14 mg/dL, protein of 101 mg/dL, and a venereal disease research laboratory (VDRL) titer of 1:4.

After desensitization, the patient was started on penicillin G 24 million units per day via continuous infusion. Concomitant glucocorticoids were not administered at the discretion of the treating physician. Two hours into his treatment, the patient developed right testicular pain with redness and swelling. Testicular ultrasound showed a 2.1 × 1.5 × 1.3-cm heterogeneously hypoechoic, solid, right testicular lesion (Fig. 1). α-Fetoprotein, human chorionic gonadotropin, and lactate dehydrogenase were all within normal limits. The patient’s testicular pain improved by the next hospital day and resolved by day 4. A urologic consultation was obtained, and the clinical syndrome was felt to be most consistent with syphilitic orchitis.

F1-8
Figure 1:
Testicular ultrasound showing a heterogeneously hypoechoic, well-circumscribed, solid testicular lesion measuring 2.1 × 1.5 × 1.3 cm.

The patient was discharged on hospital day 7 with a planned 14-day course of penicillin G. At 1-month follow-up, the patient’s visual acuity had improved slightly and repeat testicular examination showed no residual mass. At 9-month follow-up, repeat serologic testing revealed at least a 4-fold decrease in RPR titer with no recurrence of the testicular mass and continued preservation of visual acuity. The patient had since been lost to follow-up with no repeat lumbar puncture or RPR titers.

DISCUSSION

The Centers for Disease Control and Prevention has shown an increase in the incidence of syphilis in the United States during the past 10 years, especially among men who have sex with men.1 The rate of syphilis and HIV coinfection is unknown, although estimated to be 64.3% to 90% among men who have sex with men diagnosed as having syphilis.2,3

Among HIV-infected individuals, early progression to neurosyphilis and ocular syphilis is well recognized.4,5 Likewise, a diagnosis of ocular syphilis may be the initial presentation leading to an HIV diagnosis.5 Two recent reviews of ocular syphilis among both HIV-infected and HIV-uninfected individuals found that posterior uveitis was the most common finding, present in 60% of cases.6,7 Progression to retinal detachment, however, is a rare complication.8 Moreover, to our knowledge, there has been only one other reported case of a patient with retinal detachment as the presenting manifestation of syphilis and HIV coinfection.9

The diagnosis of ocular syphilis is often based on positive serologies with suggestive findings in a high-risk individual.8,10,11 Serum VDRL and RPR titers are typically elevated in ocular syphilis with a median titer of 1:128.6 An important diagnostic pitfall is the prozone phenomenon, which seems to be more common among HIV-infected individuals due to B-cell dysregulation.12 The positive FTA-abs with negative RPR on initial testing in our patient, followed by a repeat RPR titer of greater than 1:256 a few weeks later, suggests that our patient exhibited this phenomenon. Lumbar puncture is also recommended in aiding the diagnosis of ocular syphilis,8,11 although sensitivity is limited and the presence of abnormalities may be confounded by the presence of HIV in a coinfected patient.10 Among HIV-positive patients, any CSF abnormality, including pleocytosis or elevated protein, was present in only 75% of patients; VDRL was positive in only 57% of cases.6,7 Our patient, however, was noted to have a positive CSF VDRL with a lymphocytic pleocytosis, elevated protein, and low glucose, which supported the diagnosis.

Treatment of ocular syphilis is the same as neurosyphilis and consists of intravenous aqueous crystalline penicillin G for 10 to 14 days.11 The use of concomitant glucocorticoids has been recommended to avoid acute worsening of ocular symptoms from a localized Jarisch-Herxheimer reaction,8 although proof of efficacy is lacking.

Testicular lesions from syphilis are uncommon with only 13 cases reported in the past 61 years.13,14 The lesions can either present as gummas or interstitial orchitis, and cases have been described in both early and late syphilis.13–17 Clinically, syphilitic orchitis may be indistinguishable from testicular neoplasms, and most diagnoses are made after orchiectomy for presumed testicular cancer.13–16 Histologically, gummatous syphilis is a granulomatous disease with progression to central caseating necrosis, whereas interstitial orchitis is a diffuse lymphocytic-infiltrative process with areas of fibrosis.15,17 Treponemas are not commonly visualized in testicular tissue, regardless of stages of infection or HIV coinfection.13,14–17 The most sensitive and specific method of diagnosis is polymerase chain reaction–based testing of testicular tissue.15,16

In our patient, the presumptive diagnosis of testicular syphilis was made based on overall clinical presentation with onset of acute testicular pain upon initiation of penicillin therapy, suggestive of a localized Jarisch-Herxheimer reaction. To the best of our knowledge, this is the first report of a focal testicular reaction in the literature. A definitive diagnosis of syphilitic orchitis through excision or biopsy was not pursued given the high degree of suspicion for the diagnosis, with the risk of invasive procedures outweighing the benefits in the setting of rapid clinical improvement.

In summary, we describe a rare case of a man with retinal detachment and posterior uveitis as the presenting symptoms of syphilis and HIV. The acute onset of testicular pain shortly after initiation of penicillin suggested syphilitic orchitis with a local Jarisch-Herxheimer reaction in the testis. Rising syphilis rates in the era of HIV should prompt clinicians to be aware of the unusual clinical manifestations and diagnostic pitfalls of syphilis.

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