Peripheral Ulcerative Keratitis Clinical Presentation

Updated: Jun 11, 2019
  • Author: Ellen N Yu-Keh, MD; Chief Editor: Andrew A Dahl, MD, FACS  more...
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Presentation

History

Peripheral ulcerative keratitis (PUK) is frequently a manifestation of an occult systemic disease. Thus, a thorough systemic history is very important and should include chief complaint, characteristics of present illness, past medical history, family history, and a meticulous review of systems. [4]

Ocular symptoms vary, but nonspecific foreign body sensation with or without eye pain, tearing, photophobia, and reduced visual acuity are the most common symptoms for patients with PUK.

Loss of vision can occur quickly when PUK progresses.

PUK associated with RA, GPA, PAN, and RP is often linked with scleritis, and eye pain may be pronounced in these individuals. [4] PUK in patients with Mooren ulcer may also produce pain, although there is no scleral involvement.

Past medical history and review of systems helps to determine the possible underlying systemic diseases. RA, SLE, PAN, GPA, or RP may present with the following symptoms, which should be emphasized in the review of systems [12] :

  • General - Constitutional symptoms, such as chills, fever, poor appetite, recent weight loss, and fatigue
  • Skin - Rashes, nodules, vesicles, ulcer, nail changes, and periungual infarcts
  • Respiratory - Coughing, wheezing, pneumonia, and shortness of breath
  • Cardiac - Chest pain or discomfort and dyspnea
  • Gastrointestinal - Abdominal pain, nausea, vomiting, difficulty swallowing, and diarrhea
  • Musculoskeletal - Muscle or joint pain, arthritis, back pain, and limitation of motion
  • Neurologic - Headaches, seizures, psychiatric, paralysis, and numbness/tingling
  • Other systemic symptoms - Deafness, swollen ear lobes, ear infections, vertigo, and noises in ears (suggestive of RP)
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Physical

Examination should be complete and include an overview of the head (including the nose, mouth, and external ear), trunk, joints, and extremities. [4] Skin lesions should also be noted.

A complete ophthalmic examination should be performed with special emphasis on the conjunctiva, sclera, and cornea. Anterior chamber, vitreous, and fundus examinations are also important.

Patients with PUK typically present with decreased visual acuity (secondary to induced irregular astigmatism), tearing, and eye irritation with or without pain of variable duration. [5]

Bilateral disease may be present in 21% of patients. [13]

Slit lamp examination reveals a crescent-shaped destructive lesion of the juxtalimbal corneal stroma associated with an epithelial defect, stromal yellow-white infiltrates composed of inflammatory cells, and varying degrees of corneal stromal thinning (minimal to full thickness) adjacent to the limbus. [6]

In severe cases, the peripheral cornea is progressively destroyed circumferentially, and the central corneal is also damaged.

PUK accompanied by necrotizing scleritis almost always indicates the presence of a potentially lethal systemic disease. [4]

The anterior chamber should be evaluated for depth and inflammation.

A posterior segment examination is typically indicated to help determine the underlying etiology.

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Causes

The etiologies for developing peripheral ulcerative keratitis (PUK) are multiple and extensive. Connective tissue and vasculitic diseases are the major risk factors. Other disorders that can cause PUK include systemic and local infectious conditions, as well as local degenerative disorders. Although vitamin A deficiency is commonly associated with central corneal ulceration, it has also been reported to cause peripheral ulceration with or without bacterial infection of the ulcer. [14, 15]

The differential diagnosis of PUK is outlined below. [4]

  • Noninfectious conditions

    • Systemic - RA, SLE, RP, sarcoidosis, progressive systemic sclerosis, rosacea, GPA, PAN, juvenile idiopathic arthritis, [16] giant cell arteritis, Behçet disease, [17] inflammatory bowel disease, pyoderma gangrenosum, [18] metabolic conditions, nutritional deficiencies, and drug-induced side effect (checkpoint inhibitor ipilimumab) [19]

    • Local - Mooren ulcer, marginal keratitis, blepharitis (eg, staphylococcal infection, rosacea), contact lens use, chemical injury to the eyes, [20] trauma, neurotrophic and neuroparalytic causes, keratoconjunctivitis sicca, Terrien marginal degeneration, pellucid marginal degeneration, and furrow degeneration [5]

    • Surgery - Trabeculectomy, [21] cataract surgery [22]

  • Infectious conditions

    • Systemic -Shigella species, tuberculosis, syphilis, hepatitis, HIV, gonococcus, Salmonella species, and bacillary dysentery

    • Local - Herpes simplex keratitis, varicella-zoster keratitis, bacterial keratitis, fungal keratitis, and Acanthamoeba species

  • Masquerade - Malignancy - Leukemia [23, 24]

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