Category Archives: Medical

By Jean-Claude Bystryn, M.D.
Professor of Dermatology
Director of Immunofluorescence Laboratory
The Ronald O. Perelman
Department of Dermatology
New York University Medical Center

Pemphigus vulgaris (PV) can enter into remissions in which all manifestations of the disease disappear and all therapy can be discontinued. How often, and when this occurs is unclear. Review of all major studies of PV conducted during the past four decades describes remissions as occurring in less than one-third of patients.1 However, a problem with these studies is that the incidence of remissions is usually provided at only a single time point. Thus, it is unclear how long it takes for remissions to appear, how long they last and what happens when therapy is discontinued. Further complicating interpretations of the results is that the meaning of remission is often unclear. The criteria used by different investigators to define this event differ and/or are not provided. The practical outcome of this incomplete information is uncertainty about the management of pemphigus. It is unclear whether treatment simply suppresses the manifestations of the disease and must be continued permanently, or whether complete and durable remissions can be induced that permit therapy to be safely discontinued.

by Thierry Olivry, DrVet, PhD, DipACVD, DipECVD,
Associate Professor of Dermatology, Department of Clinical Sciences,
College of Veterinary Medicine, NC State University,
Raleigh, North Carolina,
and Adjunct Clinical Associate Professor of Dermatology, Department of Dermatology,
School of Medicine, University of North Carolina,
Chapel Hill, North Carolina

Autoimmune blistering skin diseases first were identified in companion animals twenty five years ago, with the description of two dogs affected with pemphigus vulgaris (PV). Two years later, the first cases of pemphigus foliaceus (PF) were recognized in canine patients. These two diseases represent the main forms of animal pemphigus being diagnosed by veterinarians.

Surprisingly, whereas the main form of pemphigus affecting human individuals is pemphigus vulgaris (PV), this entity is extremely rare in dogs with less than 50 cases being reported in veterinary medical journals. This deep pemphigus variant also has been recognized, albeit very sporadically, in rare cats and horses.

by Ernest H. Beutner, Ph.D.*
Professor of Microbiology and Dermatology,
University of Buffalo; School of Medicine and Biomedical Sciences;
Buffalo, NY

Patients with pemphigus may benefit by working with their doctors to gain a better understanding of the tests used to detect and control the disease, in some situations. Three groups of tests can help doctors diagnose pemphigus; one of these can also help to control the disease. Two groups use skin or oral tissue samples, one from light microscopy and one for direct immunofluorescence. A third group uses blood tests for the pemphigus antibodies that cause the disease; these fluctuate with disease activity. Many doctors use al three methods to check all diagnoses of pemphigus; each test gives different types of information.

By Grant J. Anhalt, M.D.
Johns Hopkins Dermatology

I will attempt to clarify what we know about the antibody response in various forms of pemphigus and how the distribution of the targeted antigens affects the location of lesions. The synthesis of this work has been proposed by Dr. John Stanley, with key published advances from Dr. Masa Amagai and Mai Mahoney, Ph.D., P. Koch and others. John Stanley refers to his concept as the “desmoglein compensation hypothesis”. The key to this hypothesis is the desmogleins (pemphigus antigens) are key adhesion molecules that keep cells attached to each other. In some areas of the body, there are two desmogleins present, and both have to be damaged to cause cell detachment – in some areas only one desmoglein may be present at some level in the skin or mucous membrane, and there only one desmoglein has to be damaged to cause cell detachment.

By David A. Sirois, D.M.D., Ph.D.
Department of Oral Medicine

Pemphigus vulgaris is a chronic autoimmune disease affecting the mucosa and skin and resulting in epithelial acantholysis, bullae formation, and chronic ulceration.1 Skin lesions of pemphigus vulgaris present clinically with typical bullae formation and ulceration. However, oral mucosal manifestations are less characteristic, typically occurring as multiple, chronic mucosal erosions or superficial ulcerations of various sizes and rarely presenting with intact bullae.2 Although pemphigus vulgaris is widely considered a skin disease, several reports of cases and case series have described it frequently as the initial, and occasionally the exclusive, site of involvement.2, 3 Thus, the unfamiliar features of oral pemphigus vulgaris could result in longer diagnostic and treatment delays than cutaneous pemphigus, which could adversely affect treatment response and prognosis.4, 5 The present study explored the natural history and diagnostic pattern of pemphigus vulgaris among 99 patients, with specific interest in the differences between oral and cutaneous pemphigus.

This is not a scientific study, but we can draw some conclusions that would tend to support the current statistics especially for PV. The one big difference would be the correlation between males and females. We had females returning surveys twice as much as males – 32% males, 68% females for PV. This fact may be due to many factors as the prevailing statistics state that this disease is equal 50/50 between men and women. From the people who have contacted the Foundation throughout the 4.5 years we have been in existence, we calculate that the statistic would be more like 60/40 female to male.

By Sergei A. Grando, MD, PhD, DSci
Professor of Dermatology
University of California Davis

The Need for Alternative Therapies for Pemphigus. In autoimmune pemphigus, systemic glucocorticosteroid treatment is life saving but may cause severe side effects. Pemphigus patients therefore need drugs that will provide safer treatment of their disease by replacing systemic use of glucocorticoid hormones such as Prednisone. Development of non-hormonal treatment is hampered by a lack of clear understanding of the mechanisms leading to pemphigus lesions. Pemphigus can be associated with myasthenia gravis, and in both diseases the autoantibodies to acetylcholine receptors are produced, suggesting a common mechanism of disease development.

by Luis A. Diaz, M.D.
Professor and Chairman Department of Dermatology Medical College of Wisconsin
Milwaukee, Wisconsin

Pemphigus foliaceous (PF) one of the major clinical variants of pemphigus, is characterized by superficial blisters and anti-epidermal autoantibodies. The epidermal antigen with which PF autoantibodies react is a desmosomal protein designated desmoglein 1 (dsg1). There are two forms of PF-a sporadic form which has worldwide distribution, and an endemic form, which has only been observed in certain rural areas of Brazil, Columbia and Tunisia.

By Dominik Ettlin, M.D., D.M.D.
Northwestern University Dental School
Chicago, Illinois

As most readers know, the National Pemphigus Foundation held its first annual meeting in Chicago on August 1, 1998. Attendees of the meeting were given a questionnaire. The purpose of this survey was to shed light on the diagnostic process of each participant, and to analyze the collected data for possible trends. The questionnaire was also posted on the Foundation's website for a few weeks. The collaboration between patients and clinicians is critical to gain more insight into the clinical and basic research aspects of the disease. I would like to take this opportunity to thank everyone who took the time to answer the questionnaire!

By Edward Tenner M.D.
Hoffman Estates, Il.

Each type of pemphigus and relate diseases has differing kinds and differing percentages of ocular involvement. Also, the treatments for these diseases have many ocular side effects. Therefore it is very important that an Ophthalmologist examines the eyes of patients with pemphigus . This is especially true at the beginning of symptoms and treatments so any eye problem can be promptly handled.