eMedicine Specialties > Ophthalmology > Cornea

Peripheral Ulcerative Keratitis: Differential Diagnoses & Workup

Author: Ellen N Yu, MD, Consulting Staff, Department of Ophthalmology, St Luke's Medical Center, Quezon City, Philippines
Coauthor(s): C Stephen Foster, MD, FACS, FACR, FAAO, 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
Contributor Information and Disclosures

Updated: Dec 8, 2008

Differential Diagnoses

Blepharitis, Adult
Keratitis, Fungal
Giant Cell Arteritis
Keratoconjunctivitis, Sicca
Herpes Simplex
Ocular Manifestations of Syphilis
Herpes Zoster
Pellucid Marginal Degeneration
HIV
Sarcoidosis
Inflammatory Bowel Disease
Tuberculosis
Keratitis, Bacterial

Other Problems to Be Considered

See Causes.

Workup

Laboratory Studies

  • Laboratory tests should focus on the suspected systemic disease.4
    • Complete blood cell (CBC) count
    • Urinalysis (UA), blood urea nitrogen (BUN), and creatinine
    • Erythrocyte sedimentation rate (ESR)
    • Rheumatoid factor (RF); positive in 80% of patients with associated RA4,14
    • Angiotensin-converting enzyme (ACE); elevated in sarcoidosis
    • Antinuclear antibodies (ANA); positive in patients with SLE and RA
    • Antibody to double-stranded DNA (anti-dsDNA); associated with SLE
    • Antibodies to small nuclear ribonucleoprotein-Sm (anti-Sm); associated with SLE
    • Antibodies to small nuclear ribonucleoproteins-RNP (anti-RNP); associated with SLE
    • Antineutrophil cytoplasmic antibodies (ANCA); C-ANCA sensitivity of 96% for active generalized WG, 67% for active regional disease, and 32% for WG in full remission after initial regional symptoms4,6,15,16,17
    • Circulating immune complex (CIC) - Raji cell assay; C-1q binding assay
    • Anti-type II antibodies; associated with RP
    • Complement - C3 and C4, CH50; consumed in patients with SLE
    • Hepatitis B surface antigen (HBsAg); present in 40% of patients with PAN
    • Fluorescent treponemal antibody-absorption (FTA-ABS) for syphilis

Imaging Studies

  • Chest x-ray and sinus CT scan to rule out WG, sarcoidosis, and tuberculosis
  • Radiographic studies of affected joints

Procedures

  • Scraping and culture of the ulcer are recommended.
  • Conjunctival resection/biopsy may be helpful in removing the limbal source of collagenases and other factors causing progressive ulceration.

Histologic Findings

In Mooren ulcer, corneal thickening occurs at the margin of the ulcer where inflammatory cells have invaded the anterior stromal layers. However, the inflammation is nonspecific, and no etiologic agent can be identified. Necrosis of the involved epithelium and stroma is seen. PUK associated with connective tissue disease and PUK associated with mild infections sometimes may appear similarly. Signs of vasculitis in the adjacent conjunctiva may be seen.18

More on Peripheral Ulcerative Keratitis

Overview: Peripheral Ulcerative Keratitis
Differential Diagnoses & Workup: Peripheral Ulcerative Keratitis
Treatment & Medication: Peripheral Ulcerative Keratitis
Follow-up: Peripheral Ulcerative Keratitis
Multimedia: Peripheral Ulcerative Keratitis
References
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References

  1. Mondino BJ. Inflammatory diseases of the peripheral cornea. Ophthalmology. Apr 1988;95(4):463-72. [Medline].

  2. Shiuey Y, Foster CS. Peripheral ulcerative keratitis and collagen vascular disease. Int Ophthalmol Clin. Winter 1998;38(1):21-32. [Medline].

  3. Messmer EM, Foster CS. Vasculitic peripheral ulcerative keratitis. Surv Ophthalmol. Mar-Apr 1999;43(5):379-96. [Medline].

  4. Foster CS, Sainz de la Maza M. Immunological considerations of the sclera. In: Foster CS, ed. The Sclera. ed. New York: Springer-Verlag; 1993:33-58.

  5. Gregory JK, Foster CS. Peripheral ulcerative keratitis in the collagen vascular diseases. Int Ophthalmol Clin. Winter 1996;36(1):21-30. [Medline].

  6. Eiferman RA, Carothers DJ, Yankeelov JA Jr. Peripheral rheumatoid ulceration and evidence for conjunctival collagenase production. Am J Ophthalmol. May 1979;87(5):703-9. [Medline].

  7. Brown SI, Grayson M. Marginal furrows. A characteristic corneal lesion of rheumatoid arthritis. Arch Ophthalmol. May 1968;79(5):563-7. [Medline].

  8. Tauber J, Sainz de la Maza M, Hoang-Xuan T, et al. An analysis of therapeutic decision making regarding immunosuppressive chemotherapy for peripheral ulcerative keratitis. Cornea. Jan 1990;9(1):66-73. [Medline].

  9. Hoang-Xaun T, Foster CS, Rice BA. Scleritis in relapsing polychondritis. Response to therapy. Ophthalmology. Jul 1990;97(7):892-8. [Medline].

  10. Watson PG, Hazelman BC. The Sclera and Systemic Disorders. Philadelphia: WB Saunders Co; 1976:241.

  11. Sangwan VS, Zafirakis P, Foster CS. Mooren's ulcer: current concepts in management. Indian J Ophthalmol. Mar 1997;45(1):7-17. [Medline].

  12. Tabbara KF. Mooren's ulcer. Int Ophthalmol Clin. Winter 1986;26(4):91-8. [Medline].

  13. Chawla B, Agarwal P, Tandon R, et al. Peripheral ulcerative keratitis with bilateral optic nerve involvement as an initial presentation of acute lymphocytic leukemia in an adult. Int Ophthalmol. Nov 16 2007;[Medline].

  14. Carson DA. Rheumatoid factor. In: Kelley WN, Harris ED Jr, Ruddy S, Sledge CB, eds. Textbook of Rheumatology. 3rd ed. Philadelphia: WB Saunders Co; 1989:664-679.

  15. Lüdemann G, Gross WL. Autoantibodies against cytoplasmic structures of neutrophil granulocytes in Wegener's granulomatosis. Clin Exp Immunol. Aug 1987;69(2):350-7. [Medline].

  16. Savage CO, Winearls CG, Jones S, et al. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis. Lancet. Jun 20 1987;1(8547):1389-93. [Medline].

  17. Nolle B, Specks U, Ludemann J, et al. Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med. Jul 1 1989;111(1):28-40. [Medline].

  18. Squirrell DM, Winfield J, Amos RS. Peripheral ulcerative keratitis 'corneal melt' and rheumatoid arthritis: a case series. Rheumatology (Oxford). Dec 1999;38(12):1245-8. [Medline].

  19. Liegner JT, Yee RW, Wild JH. Topical cyclosporine therapy for ulcerative keratitis associated with rheumatoid arthritis. Am J Ophthalmol. May 15 1990;109(5):610-2. [Medline].

  20. Miyazaki D, Tominaga T, Kakimaru-Hasegawa A, et al. Therapeutic effects of tacrolimus ointment for refractory ocular surface inflammatory diseases. Ophthalmology. Jun 2008;115(6):988-992.e5. [Medline].

  21. Chen J, Xie H, Wang Z, et al. Mooren's ulcer in China: a study of clinical characteristics and treatment. Br J Ophthalmol. Nov 2000;84(11):1244-9. [Medline].

  22. Clewes AR, Dawson JK, Kaye S, et al. Peripheral ulcerative keratitis in rheumatoid arthritis: successful use of intravenous cyclophosphamide and comparison of clinical and serological characteristics. Ann Rheum Dis. Jun 2005;64(6):961-2. [Medline].

  23. Thomas JW, Pflugfelder SC. Therapy of progressive rheumatoid arthritis-associated corneal ulceration with infliximab. Cornea. Aug 2005;24(6):742-4. [Medline].

  24. Atchia II, Kidd CE, Bell RW. Rheumatoid arthritis-associated necrotizing scleritis and peripheral ulcerative keratitis treated successfully with infliximab. J Clin Rheumatol. Dec 2006;12(6):291-3. [Medline].

  25. Lambiase A, Sacchetti M, Sgrulletta R, et al. Amniotic membrane transplantation associated with conjunctival peritomy in the management of Mooren's ulcer: a case report. Eur J Ophthalmol. Mar-Apr 2005;15(2):274-6. [Medline].

  26. Prabhasawat P, Tesavibul N, Komolsuradej W. Single and multilayer amniotic membrane transplantation for persistent corneal epithelial defect with and without stromal thinning and perforation. Br J Ophthalmol. Dec 2001;85(12):1455-63. [Medline].

  27. Tseng SC. Amniotic membrane transplantation for persistent corneal epithelial defect. Br J Ophthalmol. Dec 2001;85(12):1400-1. [Medline].

  28. Bullen CL, Liesegang TJ, McDonald TJ, et al. Ocular complications of Wegener's granulomatosis. Ophthalmology. Mar 1983;90(3):279-90. [Medline].

  29. Foster CS, Forstot SL, Wilson LA. Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis. Effects of systemic immunosuppression. Ophthalmology. Oct 1984;91(10):1253-63. [Medline].

  30. Jabs DA, Rosenbaum JT, Foster CS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol. Oct 2000;130(4):492-513. [Medline].

  31. Robin JB, Schanzlin DJ, Verity SM, et al. Peripheral corneal disorders. Surv Ophthalmol. Jul-Aug 1986;31(1):1-36. [Medline].

  32. Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. Mar 1976;60(3):163-91. [Medline].

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Further Reading

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Keywords

peripheral ulcerative keratitis, peripheral corneal ulceration, PUK, marginal corneal ulcer, corneal stroma, corneal perforation, rheumatoid arthritis, RA, vision loss, blindness

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Contributor Information and Disclosures

Author

Ellen N Yu, MD, Consulting Staff, Department of Ophthalmology, St Luke's Medical Center, Quezon City, Philippines
Ellen N Yu, MD is a member of the following medical societies: American Academy of Ophthalmology and Philippine Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, 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 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, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Institute
Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Eye Bank Association of America, Pennsylvania Medical Society, and Philadelphia County Medical Society
Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

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, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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