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JAMA Ophthalmology Clinical Challenge
June 2018

Uveal Mass, Uveitis, and Diffuse Rash in a Woman in Her 50s

Author Affiliations
  • 1Department of Ophthalmology, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada
  • 2Department of Ophthalmology, Western University, London, Ontario, Canada
JAMA Ophthalmol. 2018;136(6):704-705. doi:10.1001/jamaophthalmol.2017.5560
Case

A woman in her early 50s with no ocular history presented with a 3-week history of pain and photophobia in her right eye. She had a 1-year history of bilateral intermittent paresthesia in her feet. She also reported a 3-month history of a bilateral, erythematous, purpuric macular rash originating on her lower extremities that spread superiorly to her abdomen and chest. She works in a barn, primarily with horses, and travels to Cuba frequently for humanitarian work.

On ocular examination, her visual acuity was 20/50 OD and the intraocular pressure was 16 mm Hg. She had 1+ cells in the anterior chamber and mild, fine keratic precipitates. Superotemporally, in the anterior chamber angle, a singular, smooth, yellow-gray mass was noted (Figure, A). As seen on ultrasonographic biomicroscopy, the mass originated in the ciliary body and protruded into the peripheral iris with minimal associated vasculature (Figure, B). The results of a posterior segment examination, including optical coherence tomography, were within reference limits. Examination results of the left eye were unremarkable.

Figure.
A, Clinical photograph of the right eye demonstrating the yellow-gray tumor superotemporally in the anterior chamber angle. B, Ultrasonographic biomicroscopy of the right eye mass, demonstrating a unilobular homogeneous ciliary body tumor.

A, Clinical photograph of the right eye demonstrating the yellow-gray tumor superotemporally in the anterior chamber angle. B, Ultrasonographic biomicroscopy of the right eye mass, demonstrating a unilobular homogeneous ciliary body tumor.

Box Section Ref ID

What Would You Do Next?

  1. Obtain a punch biopsy specimen from the skin lesions

  2. Perform a ciliary body mass biopsy with limited cyclectomy

  3. Begin an empirical corticosteroid treatment regimen

  4. Order serologic tests for infectious and inflammatory disorders

Discussion
Diagnosis

Ocular gummatous syphilis

What to Do Next

A. Obtain a punch biopsy specimen from the skin lesions

The differential diagnosis for a ciliary body mass is extensive and well documented.1-3 While uveal melanomas are the most frequently encountered uveal tumors,4 ciliary body melanomas are quite rare. The acute nature of the patient’s symptoms favors an inflammatory or infectious origin. Given the patient’s upper respiratory tract, neurologic, and dermatologic symptoms, investigation into systemic causes, including granulomatosis with polyangiitis, sarcoidosis, and syphilis, is warranted. In the present case, the patient had been referred to a dermatologist for her skin lesions, which had been biopsied (choice A). Shortly after our assessment, the skin biopsy results indicated superficial and deep perivascular dermatitis with mixed cell infiltrate and prominent plasma cell components consistent with syphilis. Although VDRL or rapid plasma reagin serologic tests (choice D) would be appropriate, the high likelihood of a common origin for both the ocular and dermatologic findings makes a skin biopsy the most likely investigation to yield the correct diagnosis.

Empirical treatment with systemic corticosteroids (choice C) in the setting of a highly suspicious systemic origin would be premature. A ciliary body biopsy (choice B) ought to be reserved as the last option, should other investigations not yield a diagnosis. Last, targeted investigations specific to the patient’s signs and symptoms are preferred to a broad investigation.

Syphilis is caused by the spirochete bacterium Treponema pallidum; as with its systemic counterparts, its ocular manifestations are protean.5 Gummata are chronic granulomatous lesions that represent a failure of a delayed-type hypersensitivity reaction.6 Gummatous syphilis commonly presents in internal organs, cutaneous tissue, and bone. Although gummata have been described in a wide variety of ocular tissues,5 to our knowledge it has not been described in isolation in the ciliary body. Although gummatous syphilis is a manifestation of tertiary syphilis,7 this patient had no other signs of tertiary syphilis.

Assessment for neurosyphilis via cerebrospinal fluid (CSF) assessment ought to be performed in all patients with ocular syphilis because of both the difficulty in identifying symptoms and the higher rate of mortality associated with neurosyphilis.5,8 Spinal fluid examination not only enables diagnosis of neurosyphilis but also provides a mechanism for monitoring the effectiveness of treatment. This involves a CSF VDRL test and analyses of CSF white blood cells and CSF protein. Diagnosis may require a concurrent report to the public health department, depending on location.

Treatment of syphilis differs depending on the stage of disease and central nervous system involvement. For neurosyphilis, recommended treatment consists of intravenous penicillin G benzathine administration, 3 to 4 million U every 4 hours for 10 to 14 days.9 Other modalities have been suggested, including treatment with other antibiotic regimens, but they are currently recommended only as alternatives in patients with a penicillin allergy.10

Another important facet in the treatment of patients with acquired syphilis, particularly with ocular or neurosyphilis, HIV/AIDS coinfection, or a multitude of systemic manifestations of the disease, is a multidisciplinary approach. A collaborative effort with timely and clear communication can be important in these cases to achieve satisfactory outcomes for patients.

Patient Outcome

Following the initial report revealing the diagnosis, the patient was immediately contacted and assessed by members of the infectious diseases team, who performed a lumbar puncture that same evening. A CSF analysis revealed the presence of T pallidum, consistent with a diagnosis of neurosyphilis. Intravenous penicillin G was administered every 4 hours and continued for 2 weeks.

The patient experienced complete resolution of her systemic and ocular symptoms. The ciliary body mass receded completely, with only focal peripheral anterior synechiae present where the mass was initially observed. The remainder of her ocular examination results, including visual acuity, were within reference limits. She continues to be observed closely by her local ophthalmologist.

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Article Information

Corresponding Author: Munir M. Iqbal, MD, Department of Ophthalmology, Western University, 268 Grosvenor St, London, ON N6A 4V2, Canada (munir.m.iqbal@gmail.com).

Published Online: April 5, 2018. doi:10.1001/jamaophthalmol.2017.5560

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

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