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Peripheral Ulcerative Keratitis Clinical Presentation

  • Author: Ellen N Yu-Keh, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: May 13, 2016
 

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, WG, 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, WG, 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 centrally.

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 (Feldman, 2000).[4]

  • Noninfectious conditions
    • Systemic - RA, SLE, RP, sarcoidosis, progressive systemic sclerosis, rosacea, WG, 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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Contributor Information and Disclosures
Author

Ellen N Yu-Keh, MD Consulting Staff, Department of Ophthalmology, St Luke's Medical Center, Quezon City, Philippines

Ellen N Yu-Keh, MD is a member of the following medical societies: Philippine Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Christopher J Rapuano, MD Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director of Refractive Surgery Department, Wills Eye Hospital

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Ophthalmological Society, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, International Society of Refractive Surgery, Cornea Society, Eye Bank Association of America

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cornea Society, Allergan, Bausch & Lomb, Bio-Tissue, Shire, TearScience, TearLab<br/>Serve(d) as a speaker or a member of a speakers bureau for: Allergan, Bausch & Lomb, Bio-Tissue, TearScience.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Fernando H Murillo-Lopez, MD Senior Surgeon, Unidad Privada de Oftalmologia CEMES

Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Lijing Yao, MD, to the development and writing of this article.

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Peripheral ulcerative keratitis in the right eye of a patient with rheumatoid arthritis. Glue has been placed.
Same patient as in previous image, 1 year posttreatment.
Left eye of same patient as in previous images. Note the corneal thinning and scarring.
 
 
 
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