eMedicine Specialties > Ophthalmology > Conjunctiva

Cicatricial Pemphigoid: Differential Diagnoses & Workup

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
Coauthor(s): Rola Hamam, MD, Clinical Associate, Department of Ophthalmology, American University of Beirut; Erik Letko, MD, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School
Contributor Information and Disclosures

Updated: Dec 12, 2008

Differential Diagnoses

Neovascularization, Corneal, CL-related

Other Problems to Be Considered

Irradiation
Trauma
Progressive systemic sclerosis (scleroderma)
Toxic epidermal necrolysis
Erythroderma ichthyosiform congenita
Porphyria cutanea tarda
Epidermolysis bullosa acquisita
Linear IgA bullous disease
Paraneoplastic pemphigus
Bullous systemic lupus erythematosus
Corynebacterium diphtheriae conjunctivitis
Sebaceous cell carcinoma
Adenoviral conjunctivitis
Intraepithelial epithelioma

Workup

Laboratory Studies

  • Diagnosis of OCP is based on clinical presentation and immunohistochemical studies of the conjunctiva, which can reveal pathognomonic features of the disease.
  • 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.
  • Individuals receiving immunosuppressive agents require appropriate laboratory studies to monitor the therapy.

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.

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.

More on Cicatricial Pemphigoid

Overview: Cicatricial Pemphigoid
Differential Diagnoses & Workup: Cicatricial Pemphigoid
Treatment & Medication: Cicatricial Pemphigoid
Follow-up: Cicatricial Pemphigoid
Multimedia: Cicatricial Pemphigoid
References
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References

  1. 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].

  2. 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].

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

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

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

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

  7. 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].

  8. 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].

  9. 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].

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

  11. 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].

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

  13. 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].

  14. 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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Further Reading

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Keywords

cicatricial pemphigoid, ocular cicatricial pemphigoid, OCP, mucous membrane pemphigoid, MMP, chronic cicatrizing conjunctivitis

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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, Clinical Associate, 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, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School
Disclosure: Nothing to disclose.

Medical Editor

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

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

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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