close
  • Mega Menu Column
    • Facebook
    • Twitter
    • LinkedIn
    • Instagram
    • Donate
    • Become A Member
  • Home
  • About Us
    • Mission/Overview
    • Our Team
      • Board of Directors
      • Medical Advisors
      • Dental Advisory Council
      • Staff & Coaches
    • Financials
    • Contact Us
  • Living With P/P
    • Understanding P/P
    • All About Pemphigus (Patient Edition)
    • Diagnosis
    • Treatments
    • Physicians Referral
    • Peer Support
      • Patient Education Series
      • Discussion Forum
      • Local Support
    • My Story
    • Ask A Coach
    • Discussion Forum
  • Research
    • 2018 Pre-IID Pemphigus and Pemphigoid Symposium
    • Research Grants
    • Clinical Trials
    • Natural History Study (Patient Registry)
    • Clinically Speaking
      • All About Pemphigus (Physician Edition)
      • Pemphigoid
      • Treatments
      • Complications & Side Effects
      • Risk Factors
      • Diagnostic Testing
      • Current Consensus of Disease Outcomes
      • Prevention & Psychology
  • Get Involved
    • Healing Heroes
    • Volunteer
    • Awareness
    • Support The IPPF
      • Make A Donation
      • Healing Heroes
      • Planned Giving
      • Other Ways To Give
    • #healourskin
    • Continuing Medical Education
    • Peer Support
      • Patient Education Series
      • Discussion Forum
      • Ask A Coach
      • Local Support
    • Host A Fundraiser
    • Annual Patient Conferences
  • News
    • PemPress
    • Quarterly
    • Events
    • The Coaches Corner
  •  Facebook
  •  Twitter
  •  Instagram
  • DONATE
Pemphigus Pemphigoid Foundation logoPemphigus Pemphigoid Foundation logo
  • Menu
  • About Us
    • Mission/Overview
    • Our Team
      • Board of Directors
      • Medical Advisors
      • Dental Advisory Council
      • Staff & Coaches
    • Financials
    • Employment
    • Contact Us
  • Living With P/P
    • Understanding P/P
    • All About Pemphigus (Patient Edition)
    • Diagnosis
    • Treatments
    • Physicians Referral
    • Prescription Assistance
    • Peer Support
      • Patient Education Series
      • Discussion Forum
      • Local Support
    • My Story
    • Ask A Coach
    • Discussion Forum
  • Research
    • 2018 Pre-IID Pemphigus and Pemphigoid Symposium
    • Research Grants
    • Clinical Trials
      • What is it?
      • Should I Volunteer?
      • What are my rights?
      • Definitions
    • Natural History Study (Patient Registry)
    • Clinically Speaking
      • All About Pemphigus (Physician Edition)
      • Pemphigoid
      • Treatments
      • Complications & Side Effects
      • Risk Factors
      • Diagnostic Testing
      • Current Consensus of Disease Outcomes
      • Prevention & Psychology
  • Get Involved
    • Healing Heroes
    • IPPF Web Store
    • Advocacy
      • Becoming a Self Advocate
      • Advocacy in Action!
      • Advocacy Tools and Resources
    • Awareness
    • Support The IPPF
      • Ways to Support
      • Make A Donation
      • Healing Heroes
      • Planned Giving
      • Host A Fundraiser
      • Volunteer
    • #healourskin
    • Continuing Medical Education
    • Peer Support
      • Patient Education Series
      • Discussion Forum
      • Local Support
      • Ask A Coach
    • Annual Patient Conferences
  • News
    • PemPress
    • Quarterly
    • Events
    • The Coaches Corner

Pemphigus & Ocular Involvement: A Survey

By webadmin,

Posted by webadmin

On March 31, 2003

by Edward Tenner, M.D.

INTRODUCTION

The autoimmune bullous skin diseases, pemphigus (with major subsets pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus) and the more common bullous pemphigoid (with variant disease phenotypes of cicatricial pemphigoid and gestational pemphigoid) each may have ocular manifestations.


Diagnosis of PV is made by a characteristic clinical presentation, histological suprabasilar intraepithelial acantholysis, IgG autoantibodies on the cell surface by direct immunofluorescence testing of a biopsied lesion and the presence of circulating antiepithelial autoantibodies.1 The incidence of PV varies between 1/million in the general population to over 1/100,000 among Ashkenazi Jews in Israel. This variability correlates to a strong association of PV in individuals with selected class II MHC genes. For instance, 90% of Ashkenazi Jews with PV carry the gene allele DRB1 *0402.

New advances in characterization of the antigen and autoantibodies involved in autoimmune bullous diseases have helped elucidate the mechanism of the epithelial damage. Pathophysiologically, PV is caused by autoantibodies against desmoglein 3 (Dsg 3), a glycoprotein that localizes to the core of desmosomes. These autoantibodies cause acantholysis in the deeper suprabasal epidermis which is where Dsg 3 is mainly located. Dsg 3 is also located throughout the oral and other mucous membranes including the conjunctiva where blisters also occur in PV.2 ELISA tests for autoantibodies against desmoglein 1 (Dsg 1) and Dsg 3 are recently available that differentiate pemphigus vulgaris (anti-Dsg 3 IgG detected) from pemphigus foliaceus (only anti-Dsg 1 IgG detected), and the clinical subtypes of PV i.e. mucosal dominant (anti-Dsg 3 IgG detected) and mucocutaneous (anti-Dsg 3 IgG and anti-Dsg 1 IgG detected).

While almost uniformly fatal before the advent of corticosteroid treatment, PV can now be controlled in most patients and the mortality rate is less than 5%. Along with prednisone, an immunosuppressive drug is often needed. Azathioprine and mycophenolate mofetil are the most commonly used but in unresponsive patients cyclophosphamide, cyclosporine, or chlorambucil can be substituted.

After seeing a patient with active PV and ocular involvement, a search of the medical literature was sparse concerning ocular involvement in PV. In cooperation with the International Pemphigus Foundation a survey was undertaken to determine the prevalence and extent of ocular involvement in this rare disease.

METHODS

Questions relating to the eye were part of an International Pemphigus Questionnaire that appeared in the Foundation's website, discussion group and quarterly newsletter. A total of 158 responses were collected in 1999. Of these, 134 were from patients with PV and only those responses were considered.

RESULTS

The general disease parameters from the survey approximate what is generally known about PV, such as average age at onset in the sixth decade and equal occurrence in males and females (Table 1). Questions were framed to differentiate between prior eye problems and new ones appearing during the course of PV. New eye problems after being diagnosed with PV were reported in 62% (83 out of 134 persons). Cataract, glaucoma or both developed in 32% (43 out of 134 persons). Since most of these patients were on high dose glucocorticoid treatment, this ocular involvement most likely was treatment related and not due to PV. Although there are reports of cataract formation in pemphigus foliaceus, there is little about cataract with PV in the medical literature.

Disease related ocular involvement excluding cataract and glaucoma was noted by 40% (53 out of 134 persons), which is detailed in Table 2. It is noteworthy that blisters occurred not only on lid margins and conjunctiva but also on the eyelids themselves.3 While past reports indicate that conjunctivitis is the most common ocular involvement in PV4, the survey indicates that eyelid involvement is equally common.

CONCLUSIONS

The importance of careful ophthalmologic care for patients with PV as their disease is being treated is apparent with almost two-thirds of those surveyed having ocular problems at some time. Also because tearing and foreign body sensation due to conjunctival involvement can precede the appearance of mouth and skin lesions in PV as noted in an article by Hodak5, ophthalmologists may be among the first to see patients with this often misdiagnosed disease. Early care should minimize ocular morbidity.

REFERENCES

  1. Nousari HC, Anhalt GJ. Pemphigus & bullous pemphigoid. The Lancet 1999; 354: 667-672.
  2. Mahoney MG, Wang Z, Rothenberger K, Koch PJ, Amagai M, Stanley JR. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest.1999; 103: 461-468.
  3. Seishima M, Oyama Z, Shimizu H, Naganawa Y, Yoshimura T, Yamazaki F. Pemphigus of the eyelids. Eur J Dermatol. 2001; 11: 141-143.
  4. Ekong AS, Foster CS, Roque MR. Eye involvement in autoimmune blistering diseases. Clin Dermatol. 2001; 19: 742-749.
  5. Hodak E, Kremer I, David M, et al. Conjunctival involvement in pemphigus vulgaris: a clinical, histopathological and immunofluorescence study. Br J Dermatol. 1990; 123: 615-620.

TABLE I. General findings in 134 PV patients

Sex 79 (59%) Female 55 (41%) Male
Clinical presentation 40 (30%) Mucosal 94 (70%) Mucocutaneous
Average age, yrs 50.3 At onset of disease 55.5 At time of report

TABLE II. Ocular finding in 134 PV patients

Periocular skin & eyelid involvement 18 (13%)
Conjunctivitis 19 (14%)
Corneal problem 7 (5%)
Cataract 34 (25%)
Glaucoma 15 (11%)
Misc. (iritis, dry eyes, herpes simplex, blurred vision, blisters on eyes) 23 (17%)
Total with eye problems (some had more than one type of involvement) 83 (62%)
  • Facebook
  • Twitter
  • Google+
  • Pinterest
  • LinkedIn

Related

webadmin

Tagged:antiepithelialautoantibodiescyclophosphamidehistopathologyimmunofluorescentimmunosuppressantintraepithelialophthalmologistspathophysiologically

Post navigation

← PreviousPemphigus: A Historical Perspective
NextAutoimmune Diseases Research Plan Announced →
  • P/P Awareness Campaign
  • Disease Registry
  • Ask A Coach
  • Patients & Caregivers
  • For Medical Professionals
  • Clinical Trials
  • Treatments
  • @healourskin 18 hours

    Extended registration closes Sunday for our P/P symposium taking place prior to the @IntInvDerm18 meeting in Orland… twitter.com/i/web/status/9…

  • @healourskin 21 hours

    Pemphigus/Pemphigoid Boston Support Group is coming up on 4/28. Register for free at buff.ly/2KdPAt2

  • @healourskin April 24, 2018

    The latest Pemphigus & Pemphigoid Weekly! paper.li/healourskin/13… Thanks to @RareDiseases @jonkmanmf @AESKUTWITUS #raredisease #nord

  • @healourskin April 17, 2018

    The latest Pemphigus & Pemphigoid Weekly! paper.li/healourskin/13… Thanks to @diabetesfuna @GlobalGenes @Immunotx_papers #raredisease #nord

  • @healourskin April 11, 2018

    Save the date! 2018 Annual Patient Conference Raleigh-Durham, NC October 12-14 Hosted by Donna Culton, MD (UNC Depa… twitter.com/i/web/status/9…

  • Volunteer
  • Careers
  • Donate
  • News
  • PemPress
  • Forums
  • Contact

About IPPF

The International Pemphigus & Pemphigoid Foundation’s most important objectives are to provide patients and doctors worldwide with information about pemphigus and pemphigoid, and to provide patients and their caregivers much needed comfort and support so they can continue to live active, productive lives.
Read more »

Contact Us

International Pemphigus & Pemphigoid Foundation
1331 Garden Highway, Ste 100
Sacramento, CA 95833
United States of America

info@pemphigus.org
(855) 473-6744
(916) 922-1298

© 2017 International Pemphigus & Pemphigoid Foundation · All rights reserved.
Privacy Policy | Terms of Use | Sitemap
Website design and implementation by Uptown Studios.
en English
ar العربيةbn বাংলাzh-CN 简体中文en Englishfr Françaisde Deutschel Ελληνικάiw עִבְרִיתhi हिन्दीit Italianoja 日本語fa فارسیpt Portuguêspa ਪੰਜਾਬੀru Русскийes Españolth ไทยvi Tiếng Việt