Download Corneal Manifestations of Systemic Diseases

Corneal Manifestations of Systemic Diseases
Joseph P. Shovlin, OD, FAAO
Introduction: Assorted corneal findings can signal systemic disease.
Many of these manifestations can bring attention to some serious,
even potentially life threatening conditions. Practitioners faced with
various clinical signs affecting the cornea are forced to make an
appropriate differential diagnosis and apply adequate treatment
including needed referrals to sub-specialties within medicine.
A timely diagnosis can certainly minimize any corneal morbidity but
even impact chances of mortality. Assorted findings can be classified
into systemic causes that include metabolic disorders,
immunologic/inflammatory conditions and infectious diseases.
Generally, patients with any condition related to metabolic
disturbance are asymptomatic. Unlike patients with metabolic
disorders, patients who have an immunologic or inflammatory cause
are often symptomatic with presenting corneal signs.
Highlighted Cases
Multiple myeloma
Fabry disease
Tyrosinemia
Crack keratopathy
Thymoma (benign)
Thyroid cancer
Wilson disease
Eczema herpeticum
Corneal Cases:
Corneal Crystals
Crystals can be found in the epithelial layers and are found in
the following conditions: cystinosis, dysproteinemias,
hyperuricemia, multiple myeloma, porphyrial, monclonal
gammapathy and lipid keratopathy.
Highlight: multiple myeloma
Corneal Verticillate
Vortex keratopathy is a not uncommon finding that represents lipid
or iodine inclusions of the cornea. It can be found in Fabry
disease, assorted amphiphilic medication toxicities including drug
induced lipidosis (amiodarone, tamoxifen, suramin, chloroquine and
clofazimine to name a few) and secondary to contact lens
solution reactions. This condition can easily be confused with other
fascinations of the cornea like corneal dendritiform and
antimetabolite medication toxicity (like cytarabine) causing
degeneration of the basal epithelium and microcysts.
Highlight: Fabry disease
Corneal Dentritiform Lesions
There are many dentritiform lesions that can be seen on the
cornea. They range from true ulcerative lesion found in HSV
keratitis to non-infectious branching lesions caused by medication
toxicity. Even corneal injury can fortuitously cause a branching
dendritiform lesion during the healing response. A metabolic cause
is also illustrated.
Highlight: Tyrosinemia (Richner-Hanhart Syndrome)
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Indolent Ulcerations
Fortunately sterile corneal ulcerations are rare. Nevertheless, they
do carry potential for significant morbidity and often represent an
underlying problem. For example, sterile indolent ulcers can be
secondary to vitamin deficiencies, vernal disease, recalcitrant
herpes simplex keratitis and even crack cocaine abuse. The
substance abuse results in corneal ulceration due to the anesthetic
effect of the cocaine, the alkali burn that it causes and the irritant
smoke produced by the substance being abused.
Highlight: Crack keratopathy
Recurrent Herpes Zoster
Multiple, recurrent episodes of herpes zoster can signal a major
systemic immune problem. Immunologic suppressed or
compromised individuals are especially prone to recurrent
responses (ie. HIV positive individuals)
Highlight: Thymoma (benign)
Theodore Superior Limbic Keratoconjunctivitis
Several conditions can mimic the superior limbic
keratoconjunctivitis described by Theodore in the late 1930s.
However, once a definitive diagnosis has been made, a poorly
functioning thyroid should be suspected. Additional conditions to
consider include: sebaceous gland carcinoma, contact lens related
disease, pannus from rosacea and other skin related disease, and
infectious etiologies like chlamydia.
Highlight: Thyroidopathy with normal panel (elevated TPO)/ thyroid
CA
Kayser-Fleischer Ring (copper deposits)
Several metallic corneal deposits are possible. The location, color
and associated corneal and adnexal signs should help in making a
proper differential. For example, patients with keratoconus often
have iron deposition in the epithelium at the base of the protruding
cornea. The deep cooper deposition is highly suggestive, but not
pathognomonic for Wilson disease.
Highlight: Wilson disease
Herpes Simplex Keratitis
Primary or secondary systemic herpes can manifest itself in a rash,
fever and some fairly typical lab findings. Patients with severe
allergic immune disease can present with bilateral herpes keratitis
and persistent disciform disease. An immuno-compromised or
suppressed patient is likely to present with bilateral disease. Occult
cancers especially in the elderly are a great concern. Another
example where bilateral herpes simplex keratitis is possible is
ezcema herpeticum. Patients are in need of desensitization. Buccal
mucosal swabs may show active virus.
Highlight: Ezcema herpeticum