Cicatricial Pemphigoid Follow-up

  • Author: Anatoli Freiman, MD, FRCPC, DABD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 7, 2011
 

Complications

  • Complications of cicatricial pemphigoid include visual loss or blindness, airway stenosis, esophageal stricture, or cutaneous blistering with scarring and milia formation.
  • Keratinization of the cornea, foreshortening of the fornices, trichiasis, synechia, symblepharon, ankyloblepharon, diminished tear formation, and blindness are consequences of cicatricial pemphigoid.
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Prognosis

  • Extensive studies on the long-term outcome of patients with cicatricial pemphigoid have not been performed.
  • In general, cicatricial pemphigoid is a chronic, progressive disorder that responds poorly to therapy. Some patients may experience long-term remissions. The disease is characterized by intermittent exacerbations and waning of disease activity.
  • A recent cohort study of 35 patients with antiepiligrin cicatricial pemphigoid indicated an increased risk of malignancy that approximates that for adults with dermatomyositis. The risk is particularly high in the first year of disease.
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Patient Education

  • Be certain that patients understand the chronic nature of this disorder.
  • Help patients in identifying factors that precipitate disease activity, and instruct them to avoid conditions that exacerbate the condition.
  • Discuss the medications, including the dose, the adverse effects, and the symptoms of toxicity, with the patient.
  • Teach patients appropriate wound care.
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Contributor Information and Disclosures
Author

Anatoli Freiman, MD, FRCPC, DABD  Consulting Staff, Division of Dermatology, Women's College Hospital, University of Toronto

Anatoli Freiman, MD, FRCPC, DABD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, Canadian Dermatology Association, Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Russell Hall, MD  J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, American Society for Clinical Investigation, and Society for Investigative Dermatology

Disclosure: Genetech Grant/research funds Principle Investigator; Centecor Grant/research funds Principle Investigator; Vernallis Honoraria Consulting

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. Mar 2002;138(3):370-9. [Medline].

  2. Bernard P, Prost C, Durepaire N, Basset-Seguin N, Didierjean L, Saurat JH. The major cicatricial pemphigoid antigen is a 180-kD protein that shows immunologic cross-reactivities with the bullous pemphigoid antigen. J Invest Dermatol. Aug 1992;99(2):174-9. [Medline].

  3. Lazarova Z, Yancey K. Cicatricial pemphigoid: immunopathogenesis and treatment. Derm Ther. 2002;15:382-88.

  4. Tsubota K, Satake Y, Kaido M, Shinozaki N, Shimmura S, Bissen-Miyajima H, et al. Treatment of severe ocular-surface disorders with corneal epithelial stem-cell transplantation. N Engl J Med. Jun 3 1999;340(22):1697-703. [Medline].

  5. Daniel E, Thorne JE. Recent advances in mucous membrane pemphigoid. Curr Opin Ophthalmol. Jul 2008;19(4):292-7. [Medline].

  6. Domloge-Hultsch N, Anhalt GJ, Gammon WR, Lazarova Z, Briggaman R, Welch M, et al. Antiepiligrin cicatricial pemphigoid. A subepithelial bullous disorder. Arch Dermatol. Dec 1994;130(12):1521-9. [Medline].

  7. Egan CA, Lazarova Z, Darling TN, Yee C, Yancey KB. Anti-epiligrin cicatricial pemphigoid: clinical findings, immunopathogenesis, and significant associations. Medicine (Baltimore). May 2003;82(3):177-86. [Medline].

  8. Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. Oct 2000;43(4):571-91; quiz 591-4. [Medline].

  9. Foster CS, Sainz De La Maza M. Ocular cicatricial pemphigoid review. Curr Opin Allergy Clin Immunol. Oct 2004;4(5):435-9. [Medline].

  10. Kirtschig G, Murrell D, Wojnarowska F, Khumalo N. Interventions for mucous membrane pemphigoid/cicatricial pemphigoid and epidermolysis bullosa acquisita: a systematic literature review. Arch Dermatol. Mar 2002;138(3):380-4. [Medline].

  11. Rashid KA, Gürcan HM, Ahmed AR. Antigen specificity in subsets of mucous membrane pemphigoid. J Invest Dermatol. Dec 2006;126(12):2631-6. [Medline].

  12. Sacher C, Hunzelmann N. Cicatricial pemphigoid (mucous membrane pemphigoid): current and emerging therapeutic approaches. Am J Clin Dermatol. 2005;6(2):93-103. [Medline].

  13. Saw VP, Dart JK. Ocular mucous membrane pemphigoid: diagnosis and management strategies. Ocul Surf. Jul 2008;6(3):128-42. [Medline].

  14. Shimizu H, Masunaga T, Ishiko A, Matsumura K, Hashimoto T, Nishikawa T, et al. Autoantibodies from patients with cicatricial pemphigoid target different sites in epidermal basement membrane. J Invest Dermatol. Mar 1995;104(3):370-3. [Medline].

 
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Ocular manifestations of cicatricial pemphigoid include symblepharon, demonstrated in this photograph by the tethering of the lower lid to the cornea.
In a patient with more advanced ocular scarring, note the thickening of the lid margins, shortening of the conjunctival sulcus, and scarring. The eyelashes have been epilated after entropion developed.
By direct immunofluorescence, a linear band of immunoreactants at the epidermal-dermal junction is demonstrated by using a fluorescein-tagged antibody specific for human immunoglobulin G.
With advanced disease, ankyloblepharon (a fixed globe) develops.
 
 
 
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