eMedicine Specialties > Ophthalmology > Conjunctiva

Cicatricial Pemphigoid: Follow-up

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

Follow-up

Further Outpatient Care

  • Because relapse can occur in approximately one third of the cases, lifelong follow-up care should be continued. Patients who relapsed were found to regain disease control readily on institution of therapy and did not deteriorate to more advanced cicatrization.

Complications

  • Ocular complications of OCP include the following:
    • Corneal epithelial defects
    • Corneal stromal ulcers
    • Corneal perforation
    • Endophthalmitis
    • Glaucoma
  • Two types of lesions can occur. The most common lesion is a vesiculobullous eruption, similar to that of bullous pemphigoid. These lesions rupture spontaneously and heal without significant scarring. The second type of lesion is an erythematous localized plaque that evolves into recurring bullae, which can rupture and leave scars (ie, Brunsting-Perry dermatitis). Patients with OCP may present with skin lesions and lesions on other mucous membranes (eg, nose, mouth, esophagus, pharynx, larynx, urethra, vagina, anus).
    • Mouth involvement is the most common. Scarring of mucosa in the nose and the mouth can be debilitating. Nasopharyngeal involvement can manifest as ruptured vesicles of the nasal mucosa along with discharge, crusting, and epistaxis.
    • Patients with tracheolaryngeal lesions may present with hoarseness, dyspnea, and laryngeal stenosis. Aphonia can occur secondary to vocal cord involvement. Laryngeal stenosis and tracheal scarring with mucous accumulation may lead to fatal asphyxiation.
    • Progressive desquamative gingivitis typically results in bone loss and dental extraction.
    • Pharyngeal scarring can cause painful swallowing with subsequent malnutrition and weight loss. Patients suspected of having OCP must be questioned for the presence of dysphagia and difficulties in breathing. Endoscopic evaluation is required, if there is a possibility of tracheal or esophageal involvement.
    • Pain on defecation or rectal bleeding can manifest as a result of rectal involvement; however, esophageal involvement, typically is silent. Although the progressive scarring associated with esophageal inflammation results in esophageal strictures, which may produce dysphagia, even to the point of choking with attempted swallowing of food. As a consequence of this condition, death from asphyxiation is reported and is a major threat.

Prognosis

  • Based on the results of one study, slightly more than one third of patients receiving immunosuppressive therapy, according to the guidelines for use of immunosuppressive agents, respond to the therapy and remain free of inflammation following the cessation of therapy. Another one third of patients were free of disease activity, but they continued to receive chemotherapy because their disease had been controlled for only a short time (<1 y) or because they had a history of relapse while on therapy. Nearly one third of patients only responded partially to treatment. Inability to control inflammation and to stop progression of cicatrizing conjunctivitis was seen only in a few individuals. In these patients, intravenous immunoglobulin seems to be effective.

Patient Education

  • Patients with OCP should be educated about the disease, the potential consequences, and the therapy. It is a systemic disease affecting the eye; therefore, no topical medication can be curative.
  • It should be explained to patients that chemotherapy is currently the only available effective treatment of OCP, providing its safety by regular monitoring by an oncologist, rheumatologist, or other specialist familiar with immunosuppressive therapy.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose OCP
  • Failure to initiate treatment of OCP with systemic immunosuppressive agents
  • Failure to monitor immunosuppressive therapy and induction of adverse effects
  • Incorrect indication for surgery

Special Concerns

  • Unfortunately, most patients with OCP are diagnosed in advanced stages. Early diagnosis of OCP and early initiation of therapy are critical in preserving the visual functions. Chances to restore the vision in advanced stages of OCP are very limited and often are not successful. It is critical to perform conjunctival biopsy on each patient with chronic conjunctivitis of unexplained etiology. Ignoring the fact that OCP is a systemic autoimmune disease that requires systemic treatment can result in progression of the disease, ultimately to blindness.
 


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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