Category Archives: Medical

by Dr. Neal Halsey, Department of International Health, Johns Hopkins School of Medicine, and Dr. Noel Rose, Autoimmune Disease Research Center, Johns Hopkins University

1. What is the risk of developing smallpox?

Smallpox does not exist as a naturally occurring disease anywhere in the world. The only risk of smallpox would come from the use of smallpox as a weapon of bioterrorism. Government security experts believe that bioterrorists in several countries may have smallpox. No one knows for certain whether or not they have smallpox or if smallpox will be used as a weapon. Most infectious disease experts believe that the risk of any individual being exposed to smallpox is extremely low.

2. How good is this vaccine?

No vaccine is perfect. The smallpox vaccine provides approximately 95 percent protection against smallpox. This is about as effective as most other live viral vaccines.

3. What kinds of adverse reactions can occur?

The smallpox vaccine causes a local infection on the arm. A small group of blisters or vesicles develop associated with inflammation, swelling and tenderness that lasts for one to two weeks. About 10 percent of people develop an exaggerated reaction with more marked swelling, redness and tenderness and decreased use of the arm for a few days. Some people develop red streaks going around the arm, which resolve spontaneously, but this reaction is sometimes mistakenly assumed to be a secondary bacterial infection. Headache, fever, and feeling poorly for a few days occur frequently. About one-third of healthy young adults who were vaccinated reported missing at least one day of work, school, or participating in usual activities. More serious reactions occur much less commonly.

The vaccine virus can be transmitted from the vaccination site to some other part of the body or to people who have direct contact with a vaccinated person. The virus is transmitted on hands after touching the vaccine site. There is no evidence that the virus is transmitted through respiratory droplets or that there is any risk from being in the same room as a vaccinated person. If the vaccine virus is put on an area where the skin is broken or on a mucous membrane such as the mouth, eye, vagina, or rectum, then sores develop that are similar to the sore at the vaccination site. This can create serious problems if the viruses are placed in or around the eye or the genital area. The vaccine is not recommended for anybody with acute or chronic skin conditions that could predispose to this problem. People with eczema are at particularly high risk of developing severe reactions. Any person with eczema, atopic dermatitis, or any household member who has these conditions should not be vaccinated.

When the smallpox vaccine was given to pregnant women, the vaccine virus was sometimes transmitted to their unborn babies and caused serious infections and sometimes premature birth. When the vaccine was given to children under a year of age, they had a higher risk of developing encephalitis than older children and adults. The risk in adults is about one to two per million and this complication can lead to long-term brain damage or death.

4. Are there special risks for patients with immune disorders?

Yes, patients with immune deficiency disorders and their household contacts should not be vaccinated. Persons with defects in lymphocyte function, including people with leukemia and other forms of cancer, are at high risk of developing a serious and frequently fatal complication called progressive vaccinia. Although patients with mild defects in the immune system that do not affect lymphocyte function may not be at increased risk, there is no reason for these people to be vaccinated at this time. If there were to be outbreaks of smallpox associated with bioterrorism, these people should check with their doctors before considering smallpox vaccination.

5. I am being treated with prednisone. Should I take the vaccine?

Any person with a condition that requires the use of prednisone or other immunosuppressive agents should not receive the smallpox vaccine at this time. Although low doses of prednisone do not usually cause any problems with live viral vaccines, these persons might require higher doses of prednisone if their underlying condition became more severe. We do not have any data on what doses of prednisone might be safe for people who receive the smallpox vaccine.

6. If I have an autoimmune disease, should members of my family take the vaccine?

If you have an autoimmune disease that might require the use of steroids or other therapy that could affect the immune system, no one in your family who lives in the same household should receive smallpox vaccine because you might acquire the smallpox vaccine virus from your family member.

7. If I have an autoimmune disease, should I stay away from my place of employment if there are people there who have been vaccinated? If so, how long should I stay away?

You do not need to stay away from your place of work where people have received the smallpox vaccine. We do not have the same physical contact with people in the workplace that we have with family members. Vaccinated individuals in the workplace should have their smallpox vaccine site covered with special bandages that will markedly reduce, if not eliminate, the risk of their transmitting the virus to you. If vaccinated people take appropriate precautions with regard to care of the vaccine site, then they can continue to shake hands and have other minimal contact with other persons.

8. Are there any special precautions that I as an autoimmune disease patient should take if there is a national vaccination program?

If your disorder puts you at increased risk, you should take care to minimize direct touching contact with people who may have received the smallpox vaccine and who have an open sore that could contain the virus. If your job requires direct physical contact with people, such as people who do massage therapy, then you might consider asking clients who have been vaccinated not to come for therapy during the time when they have an open sore from the vaccine.

9. If I have an autoimmune disease that affects the skin, should I take the vaccine?

If you have active lesions from psoriasis, cutaneous vasculitis, bullous pemphigoid, Behçet's disease, discoid lupus, Mooren's ulcer, or any other skin disorder, you should not receive the vaccine. If your skin disorder is under control, you do not have any open lesions, and the vaccine is highly recommended for you because of your occupation, then you might consider receiving the vaccine after consultation with your physician. If it is not essential for you to be immunized, then we would advise against it.

10. Is there any way of treating the adverse reactions if they occur?

Some adverse reactions can be treated with a special immune globulin preparation that appears to help patients with eczema vaccinatum, severe inoculation around the eye or other sites, and possibly for progressive vaccinia. There is one antiviral drug that is available for investigational purposes, but it has a high rate of serious side effects. These drugs are available through the Centers for Disease Control and Prevention.

11. Is there a safer vaccine available for patients with autoimmune disease?

A new smallpox vaccine is currently being tested and should become available sometime during 2003. This vaccine is made from the same virus that is in the current smallpox vaccine. The new vaccine will be grown in cell culture rather than on the calf skin. Since the viruses are the same, the new vaccine will probably have the same risk of adverse reactions as the current vaccine. Scientists are working on developing safer vaccines against smallpox, but it will take at least five to ten years before these vaccines might become available.

12. Is the situation likely to change with regard to the risk of exposure to smallpox?

Almost all infectious disease experts recommend against routine vaccination of the civilian population because the risks of exposure to smallpox are very small and probably close to zero. Experts are constantly reevaluating the potential for exposure from bioterrorism. As the United Nations investigations in Iraq continue, we should learn whether or not this country has produced smallpox for possible use as a weapon. If there was one small event in a single location, this may not change the equation with regard to balancing the risks and benefits from this vaccine for the general population. Experts would contact everyone who had been exposed to be sure that they were immunized, but it would not require immunization of the general population. Smallpox vaccine can protect against smallpox even when it's administered up to four days after exposure. If someone has a known exposure to smallpox, then there are very few contraindications to using the vaccine and people with skin disorders and mild immune deficiency disorders would be immunized. If there was a confirmed large outbreak in your local community, then many experts believe that almost everyone in that community should receive the vaccine. We must always weigh the potential benefits of vaccines against the potential risks. At this time smallpox vaccination is not warranted for the general public.

American Autoimmune Related Diseases Association
22100 Gratiot Avenue
Eastpointe, MI 48021
586.776.3900
www.aarda.org

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.

By Robert Jordon, M.D.
Professor and Chairman, Department of Dermatology,
University of Texas
Houston, Texas

Historical Perspective

The term pemphigus was most likely in use in the ancient world, but the first recorded instance was by Hippocrates (460-370 BC) who described pemphigoid fever as “pemphigodes pyertoi.” Galen (AD 13 1-201) named a pustular disease of the mouth as “febris pemphigodes.” In 1637, Zacutus again uses the term “febris pemphigodes” to describe patients with blisters of short duration. DeSauvages (1760) described patients with high fever and blisters of short duration as having “pemphigus maior.” None of the above conditions is considered to be true pemphigus, as their disease was of short duration and all patients recovered.

 

By Jay Glaser, M.D.

Dr. Glaser is a board-certified internist, researcher and medical director at the Lancaster Ayurveda Medical Centers based in Sterling, MA. He can be reached at 978-422-5044. Answers to many questions about Ayurveda can be found on the Lancaster web site, www.AyurvedaMed.com, where you can subscribe to their free online newsletter, The Spirit of Health.

Sufferers of pemphigus are in a good position to aid well meaning administrators in politics, social policy, security, intelligence and defense who are currently grappling with how to re-engineer a free society immune to disruption from within or without, because this disorder recapitulates issues in domestic security. Understanding the immunology of autoimmune disorders sheds light on critical issues of individual and societal health, so we will examine immunology from both a western and eastern perspective.

By Nonhlanhla Khumalo, Dedee Murrell, Fenella Wojnarowska & Gudula Kirtschig
Archives of Dermatology March 2002 Vol 138 pages 385-389.
www.archdermatol.com

This review is different from the usual type of review you might read about bullous diseases in that by being “systematic” it was submitted ahead of time to an independent panel of editors at the Cochrane Collaboration for Evidence-Based Medicine stating that the aim was to summarize data from randomized controlled trials (RCTs) only for the treatment of BP. In addition, how those trials are to be searched for, analyzed and compared is scrutinized.

Out of all the databases only 6 RCTs were published, including 293 patients. One trial compared prednisolone at different doses: 0.75mg/ kg/day vs 1.25mg;/kg/day, and another compared methylprednisolone with prednisolone, and neither study found a significant difference between the two groups, but the patients on the higher prednisolone doses had more severe side effects.

By Vincenzo Ruocco, M.D., Sarah Brenner, M.D., and Eleonora Ruocco, M.D., from the Department of Dermatology, 2nd University of Naples, Naples, Italy, Department of Dermatology, Tel-Aviv Sourasky Medical Center, and Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel. Correspondence: Vincenzo Ruocco, MO, Department of Dermatology, 2nd University of Naples, Via Sergio Pansini, 5, 1-80131 Naples, Italy.

Introduction

In dermatology, there are typical examples of disorders related to dietary factors. The pathogenic, links between nutritional factor(s) and skin disease may be different.

Nutrient deficiency and nutrient excess are the simplest causes of specific diet-related cutaneous changes: scurvy (vitamin C deficiency) and acrodermantis enteropathica (zinc deficiency) are examples of the first type, and caronenoderma (carotene excess) is an example of the second type. Genetic metabolic defects or enzyme deficiencies, although subtle, may pave the way for the onset of diet-related skin disorders, where a toxic effect is exerted by the dietary factor(s): alcohol intake is responsible for porphyria curanea tarda, and the ingestion of choline- and lecithin-containing foods is the cause of eccnine bromhidrosis, with the typical “fishy” odor that features trimethylaminuria. More often, an immune (and complex) mechanism is involved in the pathogenesis of strictly diet-dependent skin disorders, e.g. atopic dermatitis and food-induced urticaria (related to several foods), dermatitis herpetiformis (gluten), and allergic contact dermatitis (nickel). Finally, cutaneous disorders exist where the pathogenic interference of dietary factors has repeatedly been advocated, but without convincing evidence: psoriasis, seborrheic dermatitis, and acne are the commonest examples of this type.

By Dr. Pan Meng

Pemphigus is a group of organ-specific autoimmune disorders, including pemphigus vulgaris (PV), pemphigus foliaceus (PF) and paraneoplastic pemphigus (PNP). PV is the most common disease and represents this kind of potentially life-threatening disease. It is characterized by development of blisters and erosions on skin and mucous membranes, termed acantholysis.

In China, the number of the patients with PF and PNP are lower than that of PV, perhaps because of the limitation of diagnostic methods. We only diagnose these disorders by clinical symptoms, histopathology and immuno-fluorescense. In our hospital, from 1989 to present, we have detected 32 patients with pemphigus. Among them, 28 patients were diagnosed as PV and 4 patients were PF. We found PV often occurs in middle-aged people. In two older patients, potential tumors were accompanying. Now, with the development of immunology and molecular biology, the different autoantigen in every patient can be detected. Therefore, we can redetect the patients by ELISA.

Although pemphigus is a rare disease and its incidence in China is about 0.5-3.2 per one hundred thousand people, its impact on patients is devastating. In the past, the mortality of this disease was very high and many patients died within one to two years after diagnosis. At present, with the discovery and development of glucocorticoids, for example, prednisone, patients survive. But an important problem has emerged — the side effects of this drug. After a period of taking the drug, many patients suffer from infection, higher blood pressure, diabetes and osteoporosis. Some patients died, not from the disease itself, but from the side effects of prednisone.

Therefore, how do we treat patients correctly and how do we decrease the mortality rate? We have found the combination of glucocorticoids and immunosuppressives is the best method. In the acute stage, glucocorticoids are used to control the symptoms. Then, immunosuppressives are added to avoid the rebounding when the dosage of prednisone is decreased. The most common immunosuppressives are Azathioprine, Methotrexate and Cyclophosphamide.

Also, many Chinese traditional medicines play an important role, not only in the treatment of this disease, but in alleviating the side effects of the drugs. In the theory of Chinese Medicine, the excessive heart-fire and spleen wetness-evil are the main factors of pemphigus. When they spread to the skin, pemphigus will happen. So the rule in Chinese medicine in the acute stage is to clear away heat, eliminate wetness-evil and detoxify. In the chronic stage, it is to invigorate the spleen.

In the acute stage, blisters and erosions develop on the skin and mucous membrane. Patients show red tongue with thin white fur and string-like pulse on examination by the practitioner. With the differentiation of symptoms and signs, evils of wetness stagnating within the body and the heat-evil attacking the blood were confirmed. The principal includes four parts: clear away heat, eliminate wetness, detoxify and cool the blood. The names of the main herbal medicines used: (1) Clear away heat: Long Dan Cao, Huang Chen, Bai Mao Geng, Shang Shi Gao, Zhi Mu, Da Qing Ye, Bai Hua She She Cao, etc.; (2) Eliminate wetness: Ku Sheng, Che Qian Cao, Fu Ling Pi, Sheng Yi Mi, etc.; (3) Detoxify: Liu Yi San, DA Qing Ye, Lian Qiao, etc.; (4) Cool the blood: Sheng Di, Dan Pi, Chi Shao. The typical prescription is Long Dan Cao 10g, Huang Chen 10g, Bai Mao Geng 15g, Sheng Di 15g, Sheng Shi Gao 20g, Zhi Mu 10g, Liu Yi San 30g, Fu Ling Pi 10g, Sheng Yi Mi 30g, Di Fu Zi 20g, Bai Hua She She Cao 30g. It is concocted in water for oral doses, and can also be used on the lesion directly.

In the chronic stage, lesions become dry and erosions are cured. Patients feel itching on the skin. The signs of the tongue, the fur and the pulse become better than before. The main treatment is adding some other herbal medicine to alleviate itching, for example, Di Fu Zi, Bai Xian Pi, She Chuang Zi, etc. The typical prescription is Long Dan Cao 15g, Huang Chen 10g, Bai Mao Geng 20g, Sheng Di 15g, Liu Yi San 30g, Da Qing Ye 30g, Dan Pi 15g, Dong Gua Pi 20g, Ze Xie 15g, Zhu Ling 30g, Fu Ling Pi 30g, Sheng Mi Ren 30g, Ku Sheng 15g, Di Fu Zi 25g, Bai Hua She She Cao 30g, Chuan Cao Xian 15g, Bai Xian Pi 20g, Sheng Bai Shu 10g, Ma Chi Jian 30g.

We must point out that the treatment of Chinese tranditional medicine is an accessorial treatment in this severe disease. Its function is to reduce the dosage of the drugs and to facilitate the decrease of the drugs, further to decrease the side effects of the drugs. Besides these herbal medicines, many Chinese medicines also can be used to increase the immune system of the patients. For example, Lei Gong Teng, An Beng Feng, Lu Qui, and so on.

The goal of therapy is not to continue all medical treatment, but to improve the patients' quality of life. I hope traditional Chinese medicine will be of some benefit in achieving this goal.

By Professor Martin M Black, MD

Pemphigus and its variants are rare autoimmune disorders characterized by loss of cell to cell cohesion between keratinocytes leading to intra-epidermal blistering. In all types of pemphigus, antibodies are directed against antigens in the intercellular substance between keratinocytes and in a substantial number of active cases, these pemphigus antibodies can be detected in the general blood circulation.

Pempigus vulgaris (PV) is characterized by flaccid blistering and erosions of the skin and mucous membranes. Involvement within the mouth may often precede the skin erosions and may persist even long after skin lesions subside. It is therefore important to remember that involvement of the oral cavity may take the patient to see dental surgeon, rather than a dermatologist in the first instance. However, in pemphigus foliaceus (PF) the blistering tends to be more superficial than in the vulgaris form of pemphigus and the mucous membrane areas are not involved.

For many years, London, one of the largest cities in the world, has been truly international, with large populations of different ethnic groups living together within a 50 mile radius. This multi ethnicity provides an ideal opportunity to study the epidemiology of pemphigus and provide information on ethnic groups and numbers of involvement. We have recently surveyed our 140 patients of pemphigus who attend our St. John’s Institute for Diseases of the Skin. In our group, the male to female ratio was 1:1.12 (77 F, 63 M) and the mean age of disease onset was 44 years. This of course, is in the prime of adult life and has important economic consequences for patient sufferers, particularly if the disease is severe and the treatment likely to be longstanding. In our patients, the ethnic break-up of our group was British 51 (36.4%), Asian (Indian subcontinent) 46 (32.8%). This is quite a high figure and corroborates other evidence that pemphigus is much more common in patients living in the Indian subcontinent countries. Of Afro-caribbean countries, 15 (10.7%) had pemphigus, Middle-East 12 (8.5%) and curiously, Jewish 9 (6.4%) is rather low, as all the text books state that pemphigus is much more common in those of Jewish ancestry. Others of mixed ethnicity are fewer in number and comprise 2 Greeks and 2 Chinese. This evidence certainly would indicate the genetic factors and have an important role in predisposing individuals to developing pemphigus. It is therefore an opportunity for us to develop this theme further and we will be doing this over the next few years, looking at the genetic haplo types.

For over 25 years, our immunodermatopathological laboratory at our Institute has specialized in diagnosis of auto-immune bullous diseases. We have developed considerable experience with immunofluoresence techniques to detect the presence of antibodies in the skin by the direct method and in the serum by indirect methods. It is now well known that the PF antigen is a transmembrane glycoprotein called desmoglein 1 (Dsg1) and the PV angiten is termed desmoglein 3 (Dsg3). These desmogleins are adhesion molecules belonging to the cadherin family of cell adhesion substances and are very important in keeping the covering of our skin together.

A recent innovation has been the introduction of an antigen specific ELISA test in the diagnosis of pemphigus. The patient’s serum is tested on ELISA plates pre-coated with recombinant proteins of the ectodomain of Dsg1 or Dsg3 antigens (Medical and Biological laboratories Co, Ltd, Nagoya, Japan). Thus specific antibodies directed against Dsg1 or Dsg3 antigens can be detected by this technique.

It was seen that 61% of patients with PV have antibodies to Dsg 1 in addition to Dsg3, and presence of both the antibody types was associated with severe cutaneous and mucosal involvement, while presence of only Dsg3 autoantibodies was associated with pemphigus limited to mucosal surfaces (mainly oral). The proportion of Dsg1 positive PV patients was higher in the Asian ethnic group when compared to their British counterparts. The severity of the skin and oral disease is influenced by the quantities of Dsg1 and Dsg3 antibodies present in a patient.

Conclusion

Whether ELISA plate techniques will eventually overtake immunofluoresence in the diagnosis of pemphigus and related diseases is too early to say, but they are an important advance and enable large numbers of samples to be read quite quickly. I am sure those of you who are interested in pemphigus will see much more on this in the future about diagnostic techniques. Clearly, accurate diagnosis will ultimately lead to the potential of good targeted therapies.

The word “auto” is the Greek word for self.    If a person has an autoimmune disease, the immune system mistakenly attacks itself, targeting the cells, tissues, and organs of a person’s own body. 

by Christopher D. Saudek, M.D.,
Professor of Medicine, Johns Hopkins University School of Medicine,
President, American Diabetes Association (July 2001).

Steroids are commonly used in medicine, and their effects on blood sugar are often seriously underestimated. To give the bottom line first, steroids seriously increase the blood sugar level of people who have diabetes, and they cause or uncover diabetes in many people who don’t yet have it.

While there are several kinds of steroids, such as the ones used in muscle building (“anabolic or androgenic steroids”), in this discussion we are talking about the group called corticosteroids or glucocorticoids, exemplified by the medications hydrocortisone, prednisone or dexamethasone.