Ophthalmologic Manifestations of Cicatricial Pemphigoid Workup

  • Author: C Stephen Foster, MD, FACS, FACR, FAAO; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Aug 23, 2011
 

Laboratory Studies

  • Diagnosis of OCP is based on clinical presentation and immunohistochemical studies of the conjunctiva, which can reveal pathognomonic features of the disease.[3]
  • Currently, no specific laboratory assays are available to diagnose or monitor the activity of OCP; however, such assays are being developed. In one study, decreased serum levels of interleukin 6 and increased serum levels of tumor necrosis factor alpha were described in patients with active OCP, but the use of these tests is not common in clinical practice.[4]
  • Individuals receiving immunosuppressive agents require appropriate laboratory studies to monitor the therapy.
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Procedures

  • Conjunctival biopsy
    • Definitive diagnosis of OCP is made by demonstration of linear deposition of immunoreactants (eg, IgG, IgA, complement component C3 or C4) at the BMZ of biopsy specimen of inflamed conjunctiva using immunofluorescent or immunoperoxidase technique.
    • Other histologic techniques, such as hematoxylin and eosin staining, periodic-acid Schiff (PAS), and Giemsa staining, are not diagnostically specific.
    • Only experienced laboratory technicians should process conjunctival tissue to obtain the highest possible diagnostic yield and sensitivity. A negative or inconclusive biopsy result may be secondary to poor biopsy technique or poor handling of the specimen.[5]
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Histologic Findings

Hematoxylin and eosin staining shows the conjunctiva infiltrated with neutrophils, macrophages, and Langerhans cells. PAS goblet cells are decreased or absent in patients with advanced OCP. Patients with active OCP have excess mucus production and strands of mucuslike material in the inferior fornix.

Observations with scanning and transmission electron microscopy indicate mucus present on the surface of the conjunctiva, even though goblet cells are not seen. Giemsa stain results show that the total mast cell number and ratio of connective tissue mast cells to mucosal mast cells are significantly higher than in normal conjunctiva.

The deposition of IgG, IgA, C4, or C3 is highlighted by fluorescein or rhodamine-labeled antibodies, which are directed against immunoglobulins and complement components. The diagnostic sensitivity of immunofluorescence alone is approximately 50-52%.

Immunoperoxidase technique is required when immunofluorescence study findings are negative, yet the clinical presentation strongly suggests OCP. The immunoperoxidase technique is approximately 1,000 times more sensitive than immunofluorescence. Immunoperoxidase can detect deposition of immunoreactants at the BMZ in smaller amounts. The diagnostic sensitivity of immunoperoxidase is 83%, an increase of 31% compared to immunofluorescence technique.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

Rola Hamam, MD  Assistant Professor, Department of Ophthalmology, American University of Beirut

Rola Hamam, MD is a member of the following medical societies: American Academy of Ophthalmology and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Erik Letko  MD, Corneal Consultants of Colorado

Disclosure: Nothing to disclose.

Specialty Editor Board

Jerre Freeman, MD  Founder and Chairman, Memphis Eye and Cataract Associates; Clinical Professor, Department of Ophthalmology, University of Tennessee Health Science Center College of Medicine

Jerre Freeman, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, and Tennessee Medical Association

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

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 Institute

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

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching; EyeGate Pharma Consulting; Inspire Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching; Bausch & Lomb Consulting fee Consulting

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.

References

  1. Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc. 1986;84:527-663. [Medline].

  2. Chan RY, Bhol K, Tesavibul N, et al. The role of antibody to human beta4 integrin in conjunctival basement membrane separation: possible in vitro model for ocular cicatricial pemphigoid. Invest Ophthalmol Vis Sci. Sep 1999;40(10):2283-90. [Medline].

  3. Nguyen QD, Foster CS. Cicatricial pemphigoid: diagnosis and treatment. Int Ophthalmol Clin. Winter 1996;36(1):41-60. [Medline].

  4. Cordero Coma M, Yilmaz T, Foster CS. Tumour necrosis factor-alpha in conjunctivae affected by ocular cicatricial pemphigoid. Acta Ophthalmol Scand. Nov 2007;85(7):753-5. [Medline].

  5. Power WJ, Neves RA, Rodriguez A, et al. Increasing the diagnostic yield of conjunctival biopsy in patients with suspected ocular cicatricial pemphigoid. Ophthalmology. Aug 1995;102(8):1158-63. [Medline].

  6. Hall VC, Liesegang TJ, Kostick DA, et al. Ocular mucous membrane pemphigoid and ocular pemphigus vulgaris treated topically with tacrolimus ointment. Arch Dermatol. Aug 2003;139(8):1083-4. [Medline].

  7. Foster CS, Wilson LA, Ekins MB. Immunosuppressive therapy for progressive ocular cicatricial pemphigoid. Ophthalmology. Apr 1982;89(4):340-53. [Medline].

  8. Foster CS, Ahmed AR. Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid: a preliminary study. Ophthalmology. Nov 1999;106(11):2136-43. [Medline].

  9. Sami N, Letko E, Androudi S, et al. Intravenous immunoglobulin therapy in patients with ocular-cicatricial pemphigoid: a long-term follow-up. Ophthalmology. Jul 2004;111(7):1380-2. [Medline].

  10. Foster CS, Chang PY, Ahmed AR. Combination of rituximab and intravenous immunoglobulin for recalcitrant ocular cicatricial pemphigoid: a preliminary report. Ophthalmology. May 2010;117(5):861-9. [Medline].

  11. Daoud Y, Amin KG, Mohan K, Ahmed AR. Cost of intravenous immunoglobulin therapy versus conventional immunosuppressive therapy in patients with mucous membrane pemphigoid: a preliminary study. Ann Pharmacother. Dec 2005;39(12):2003-8. [Medline].

  12. Heiligenhaus A, Shore JW, Rubin PA, et al. Long-term results of mucous membrane grafting in ocular cicatricial pemphigoid. Implications for patient selection and surgical considerations. Ophthalmology. Sep 1993;100(9):1283-8. [Medline].

  13. Sainz de la Maza M, Tauber J, Foster CS. Cataract surgery in ocular cicatricial pemphigoid. Ophthalmology. Apr 1988;95(4):481-6. [Medline].

  14. Neumann R, Tauber J, Foster CS. Remission and recurrence after withdrawal of therapy for ocular cicatricial pemphigoid. Ophthalmology. Jun 1991;98(6):858-62. [Medline].

  15. Foster CS, Neumann R, Tauber J. Long-term results of systemic chemotherapy for ocular cicatricial pemphigoid. Doc Ophthalmol. 1992;82(3):223-9. [Medline].

  16. Saw VP, Dart JK, Rauz S, et al. Immunosuppressive therapy for ocular mucous membrane pemphigoid strategies and outcomes. Ophthalmology. Feb 2008;115(2):253-261.e1. [Medline].

 
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Ocular cicatricial pemphigoid, stage II. Note the fornix foreshortening.
Ocular cicatricial pemphigoid, stage III. Note the symblepharon.
Ocular cicatricial pemphigoid, stage IV. Note the ankyloblepharon and ocular surface keratinization.
Corneal neovascularization with ulceration and stromal thinning after persistent epithelial defect in a patient with ocular cicatricial pemphigoid.
 
 
 
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