Ophthalmologic Manifestations of Cicatricial Pemphigoid Follow-up

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

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.[14]
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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.
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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.[15]
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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.[16]
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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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