A 58 Year Old Woman with Vision Loss, Headaches, and Oral Ulcers.pdf

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Case Records of the Massachuset ts Gener al Hospital

well to prednisone and biologic agents such as
infliximab.1 Third, the presence of a palmar rash
does not fit with a diagnosis of Behçet’s disease.
Fourth, retinal vasculitis, which is characteristic
of Behçet’s disease, was absent in this patient
even when chorioretinitis was present. Finally,
although the test for HLA-B51 was positive, this
test has minimal clinical usefulness in patients
from North America.2

The first clinically significant eye disease that
occurred in this patient was scleritis, which is a
rare condition. The sclera is the white tunic that
surrounds the eye; biopsy of the sclera is rarely
performed because of the potential to perforate
the globe. Scleritis can be a localized manifestation of a systemic vasculitis (Table 2).3,4 Although
eosinophilic granulomatosis with polyangiitis
does not commonly cause scleritis, it should be
considered in this patient, especially since she
had a history of asthma. However, the presence
of a rash, the absence of eosinophilia, the lack
of response to immunosuppression, and the eventual retinitis and central nervous system (CNS)
disease all argue against this diagnosis.
Scleritis is often divided into types. Posterior
scleritis is the least common type and the most
difficult to diagnose because the posterior sclera
can be inflamed without producing any redness.
Posterior scleritis is a possible cause of the retinal changes that were noted on examination in
this patient. The usual way to assess whether
posterior scleritis is present is to obtain ultrasound images of the eye. Although results of
ocular ultrasonography have not been provided,
posterior scleritis would not account for some of
the major features of this patient’s illness, such
as CSF pleocytosis and palmar rash.

The cause of uveitis is associated with the
location of the inflammation in the uveal tract.
Scleritis occasionally causes uveitis, but it would
be unusual for the scleritis and uveitis to occur
at different times, as in this patient. Diseases
that tend to cause both scleritis and uveitis are
listed in Table 2. The only one of these diseases
that would typically cause retinitis is Behçet’s
disease, which is an unlikely diagnosis in this
case, as discussed previously.
Causes of uveitis can be divided into broad
categories, including infection, immune-mediated
disease, reaction to medication, trauma, and syndromes that masquerade as uveitis, such as cancer (usually a B-cell lymphoma) (Table 2). Nothing in this patient’s history suggests exposure
to a medication that might cause uveitis, such as
intravenous bisphosphonate5 or an immune checkpoint inhibitor,6 and there is no history of trauma.
B-cell lymphoma must be considered in this patient because it can cause retinitis, bilateral uveitis, and CSF pleocytosis.7 It can progress slowly,
over a period of years, and it would not be adequately treated with immunosuppression. It usually occurs in association with disease in the
brain. However, imaging studies did not show
findings suggestive of lymphoma, and lymphoma
typically does not cause a rash or scleritis.

In a Case Record published in 2002, there was a
scholarly discussion of uveomeningitis.8 Potential causes of combined uveitis and CNS disease3
include syphilis, primary CNS lymphoma, the
Vogt–Koyanagi–Harada syndrome, and multiple
sclerosis. The Vogt–Koyanagi–Harada syndrome
causes uveomeningitis but is not associated with
palmar rash; it also causes characteristic serous
elevations of the retina and not scleritis. Multiple sclerosis would not account for the rash or
scleritis, and patients with multiple sclerosis typiThe uvea consists of the iris, ciliary body, and cally have fewer leukocytes in the CSF than did
choroid. Uveitis is diagnosed when there is in- this patient. Primary CNS lymphoma has been
flammation of the uvea, such as chorioretinitis, ruled out, but syphilis remains a possibility.
or when leukocytes are present in the anterior
chamber of the eye or in the vitreous humor. Palmar Rash and Eye Disease
Uveitis is classified according to the inflamed Another approach to the differential diagnosis
portion of the uvea; types include anterior, inter- of this patient’s illness would be to relate the
mediate, and posterior uveitis, as well as panu- palmar rash to the eye disease. Most rashes
veitis. On the basis of the findings on examina- spare the palms. Causes of rash that involves the
tion, this patient had panuveitis.
palms include syphilis, psoriasis, reactive arthri-

n engl j med 380;11 nejm.org  March 14, 2019

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