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Pemphigus is an autoimmune condition of the skin. Its unique geographic distribution provides clues to its origin and treatment options. Certain regions of Brazil, Colombia and Tunisia have a relatively high incidence of pemphigus in young adults and children. A 14-year study in Brazil showed that environmental triggers may play a key role.
A variety of pemphigus called Fogo selvagem (FS) is endemic to a subtropical area of Brazil from 5 to 25 degrees south latitude, at an altitude of 1,600 to 2,600 feet. FS patients are usually outdoor agricultural workers, living in close proximity with rodents, bedbugs, Reduvid bugs, black flies and sand flies. New cases of FS are most common at the end of the rainy season, when insect multiplication is highest. Leishmaniasis, Onchocerciasis and Chagas disease share the same regional distribution. These diseases are all believed to be transmitted by insects.
Pemphigus is not an infectious disease, but researchers speculate that the saliva of blood-sucking insects may trigger the condition in genetically susceptible individuals. Patients who move to an industrialized city often see an improvement in their condition. Thus, removing the environmental trigger may be an effective treatment.
Once triggered, the immune system produces antibodies to specific proteins that are important for cell adhesion in the skin. Since the skin is readily accessible to researchers, the molecular mechanisms of pemphigus are well characterized.
Without treatment, pemphigus has a poor prognosis. According to Brazilian researchers, it was once said that if the patient survived for more than a year, then the diagnosis of pemphigus was wrong. The introduction of steroids in the 1950's offered a much better future to patients. Additional drug treatments include azathioprine, cyclophosphamide, methotrexate, mycophenolate mofetil, and cyclosporine. As with other autoimmune conditions, plasmapheresis and immunoadsorption offer some hope for cleaning out the antibodies that are attacking the skin. Intravenous immunoglobulin (IVIg) is also helpful for some patients. The International Pemphigus and Pemphigoid Foundation offers support for patients and families. Services include referrals to expert doctors, news and meetings. The 2010 annual meeting is scheduled for April 30 – May 2, in Philadelphia, Pennsylvania.
Identification and elimination of the environmental trigger remains a goal of pemphigus research. In addition, 19 clinical trials are currently underway for new drug therapy options.
References:
Culton DA et al, “Advances in Pemphigus and its Endemic Pemphigus Foliaceus (Fogo Selvagem) Phenotype: A Paradigm of Human Autoimmunity”, J Autoimmun. 2008 Dec; 31(4): 311-324.
International Pemphigus and Pemphigoid Foundation:
http://www.pemphigus.org/
Clinical Trials for Pemphigus:
http://clinicaltrials.gov/ct2/results?term=pemphigus
Linda Fugate is a scientist and writer in Austin, Texas. She has a Ph.D. in Physics and an M.S. in Macromolecular Science and Engineering. Her background includes academic and industrial research in materials science. She currently writes song lyrics and health articles.
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Interesting article! I live in Utah and I found two Kissing Bugs in my work area within the last few weeks. I was curious about Chagas Disease and found this article. While I don't believe the parasite can live this far north, I am wondering about Pemphigus, what it looks like, how long it lasts, and is it mistaken for other autoimune conditions. FYI: I have been suffering from a skin condition that I was told was both like eczema and psoriasis. My doctor prescribed a strong steroid cream which eased the condition considerably. Later I learned that if I eat foods that have iodine in them my condition, extremely serious, almost dissappears. I wonder if my condition is related to the Pemphigus condition. Iodine is displaced in the thyroid by other chemicals like bromine and chlorine. If enough bromine enters the system, iodine is not available for proper thyroid and immune system function. If the saliva of these bugs and other substances that ripen conditions for Pemphigus also block iodine from the thyroid the skin conditions that result (blisters, pus, thickening, etc.) may be related to my own condition. For iodine control I do not drink any citrus sodas that contain Brominated Vegetable Oil or bromines of any kind (Mt Dew, Squirt, and others...). I also do not use mouthwash or toothpastes (my teeth and gums are very healthy without them). For iodine suplementation I simply eat a sheet of roasted seaweed each day. Eggs have some iodine, but because iodine is in such a small amount it's not consistent. I do not boil my seaweed because the iodine has a tendency stick to the sides of the pan (iodine is an ion) or go out with the steam. I may consider a controlled dose in the future, but for now, seaweed is all I need. Thank you for the interesting article!
December 30, 2009 - 12:04pmThis Comment