Don't think that it's just a big fire! This immune skin disease is severely fatal
If you break your mouth for a long time, don't think it's just too much! The doctor pointed out that if the oral cavity is repeatedly broken, and the skin on the other side of the body also appears blisters, it may be a rare immune skin disease "pemphigoid", which may be fatal and must not be ignored.
"Although this type of disease is rare, it is a major injury. Patients are attacked by autoimmune attacks because of the connection between skin cells. If they are broken, they may break the skin. In the past, when someone went to the emergency department, they were all blisters and broken skin. I have no skin and I live directly in the burn ward."
Zhu Jiayu, the skin department of National Taiwan University Hospital, pointed out that pemphigus is a very rare disease. It is estimated that there will be no more than one hundred people in Taiwan in a year. The disease is caused by the patient's autologous B cells producing antibodies that attack the bridge between the skin cells—the interstitial protein—which is responsible for attaching the cells to the cells, like cement stacking bricks. together. When the protein is attacked, the epidermal cells become a piece of brick, which can no longer be hand-joined together, so that the patient's skin can be broken when it is touched, and blisters are not used.
"Many people in this class thought at first that they were just angry or accidentally biting. They didn't care. Until the pain couldn't stand, they went to the dentist or the otolaryngology to see a doctor. One coat. But then I found that the problem was not solved. I broke the skin after one or two weeks, and then I started to see the doctor everywhere. It is estimated that it will take at least two or three months from the onset of symptoms to the confirmation of pemphigus."
"There were patients who had lost their mouths and almost had difficulty eating. They lost eight or nine kilograms in just three months." Zhu Jiayu pointed out that dermatologists or dentists generally understand the disease and therefore see this oral cavity. Patients who repeatedly break the skin and have blisters on their bodies may wonder if it is the disease and further referral to the treatment of a large hospital!
Zhu Jiayu explained that the main reason is that these patients must be treated with high doses of steroids, so they will be referred to large hospitals.
This biologic was originally used to treat B-cell lymphoma, a targeted drug, and was later used to treat rheumatoid immune diseases such as rheumatoid arthritis and vasculitis. In 2007, a research team in France used it to treat pemphigus, and the response was very good. The National Taiwan University Hospital was also used in 2008, mainly for patients with poor response to steroid therapy or steroid-dependent treatment.
"Patients with poor steroid response are you who have steroids, but the blisters in the patients continue to emerge. As for the steroid-dependent patients, steroids are worse if they don't hit the disease." "But you can't be infinite." He has been applying high doses of steroids all the time! After all, there are still serious side effects, in addition to the common face swelling, buffalo shoulders, stomach bleeding, high blood sugar, high blood pressure, high blood fat, and even patients are young because High-dose steroids cause hip necrosis and have to change artificial joints. They also die from severe sepsis."
As for biological agents, by killing B cells, it is prevented from continuing to produce antibodies that can damage the intercellular connections. With the experience of the National Taiwan University Hospital, patients can be relieved. It is worth noting that although the patient may still relapse, as long as the treatment is given again, most patients can be relieved. "And if you do more treatments, you may not even relapse."
Zhu Jiayu explained, "Every treatment is like annihilating a group of an army (B cells), and then recruiting recruits (the body makes new B cells), although there may still be some rebellion, but not as much as last time. Therefore, when the annihilation once and for all, the rate of renegade will become lower and lower."
Although it has been in clinical use since 2007, Taiwan officially passed the indications this year until the US Food and Drug Administration officially passed the treatment for pemphigus last year. Zhu Jiayu explained, "The main reason is that there are too few patients with pemphigus. It is not easy to conduct relevant clinical trials. The previous clinical trials were completed by many hospitals across Europe."
Because in the past, there were patients who did not want to receive steroid treatment, stopped taking drugs, and even applied "pearl powder" to the wound to repair the wound. As a result, when the whole body smelled bad and went to the doctor, the doctor still had a hand. The foreign body on her wound was cleared, not only pain, but the last patient died of sepsis.
Therefore, Zhu Jiayu also reminded me that if you find the blisters and broken skin that will not heal in the long-term and the body, you should seek professional doctors for treatment! Don't listen to remedies and delay medical treatment.
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