Pemphigus is not contagious – not even blood to blood. Pemphigus is treatable and manageable. Pemphigus patients are not alone.
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What are pemphigus and pemphigoid?
Pemphigus
Pemphigus is a group of rare autoimmune blistering diseases of the skin and/or mucous membranes. Pemphigus is chronic and progressive and characterized by blisters and ulcers on the skin and mucous membranes. The oral mucosa is often the first site of involvement. Typically patients will have had multiple oral ulcers (broken blisters) that persist for weeks to months. PV affects the oral mucosa in nearly all cases (>80%) and affects the oral mucosa first in the majority of cases, 사실 환자에서 유일한 사이트 수.
소수만 피부 erosions 함께 발표할 예정 이다. 피부 관련 된은 일반적으로 상단의 가슴, 뒤로, 두 피, 그리고 얼굴, 하지만 병 변 신체의 어떤 부분에 발생할 수 있습니다.. 참고 피부 지배적인 양식 Dsg1 autoantibody 프로필 명단 (마찬가지로 pemphigus foliaceus), 반면에 mucosally 지배적인 양식 명단 Dsg3 autoantibody 프로 파일. 피부와 점 막의 혼합된 패턴 매니페스트 혼합된 Dsg1 및 Dsg3 autoantibody 프로 파일. 주 개월 이상 조건 진행.
손톱 주위에 자주 간과 사이트 포함 됩니다. (고통 명단, 레드, 그리고 부), 인 두와 후 두 (삼 키 고 쉰에 통증), 그리고 코 구멍 (비 강 혼잡 하 고 살 벌 한 점액 방전, 아침에 코를 불고 때 특히 눈에 띄는). 만큼 49% of patients were shown to have laryngeal and nasal involvement.
Antibodies that attack one’s own cells are called autoantibodies. The part of the cells that are attacked in pemphigus are proteins (desmogleins) which act as the glue that attaches adjacent skin cells, keeping the skin intact. When autoantibodies attack desmogleins, the cells become separated from each other. The skin virtually becomes unglued. This causes burn-like lesions or blisters that do not heal naturally. In some cases, these blisters can cover a significant area of the skin.
The disease appears to affect people across racial and cultural lines, so it’s not possible to predict who may get pemphigus. Although fairly common in adults, pemphigus is rare in children. In children, it is most often caused by placental transmission of IgG autoantibodies in utero from a woman who has pemphigus.
If left untreated, the erosions and bullae of pemphigus vulgaris gradually spread, involving an increasing surface area, and can become complicated by severe infections and metabolic disturbances.
Before the advent of corticosteroids, pemphigus had a high fatality rate, with approximately 70+% of patients dying within a year.
With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with pemphigus often resolves after several months. Most patients treated for pemphigus will enter a partial remission within 2 to 5 years. In a longitudinal study of outcome in 40 patients with pemphigus vulgaris, 45% entered a complete and long-term remission after 5 years and 71% after 10 years.
Pemphigoid
Pemphigoid is a group of sub-epidermal, blistering autoimmune diseases that primarily affect the skin, especially the lower abdomen, groin, and flexor surfaces of the extremities. Here, autoantibodies (anti-BPA-2 and anti-BPA-1) are directed against the basal layer of the epidermis and mucosa. The condition tends to persist for months or years with periods of exacerbation and remission. Localized variants of the condition have been reported, most often limited to the lower extremities and usually affecting women. There are two predominant types of pemphigoid: mucous membrane pemphigoid (MMP) also called cicatricial pemphigoid, and bullous pemphigoid (BP). Pathogenesis and management are quite different for these conditions. Scar formation in mucous membrane pemphigoid can lead to major disability.
There are two major types of pemphigoid, 조기 진단이 중요. 비록 거기에 유전 및 질병의 발생률이 높은 일부 그룹 있을 수 있습니다, 인종과 문화적 라인에 걸쳐 사람들에 영향을 미치는 질병이 나타납니다., 그래서 누가 pemphigoid를 얻을 수 있습니다 말할 수 또는 왜. 치료는 가능, pemphigoid과 함께 생활 하는 것에 대 한 도움말은, 영양 및 간 병에 대 한 정보를 포함 하 여. 보호자, 환자, 의사는 환자의 불안 수준 및 BP와 대처 능력 논의 해야, BP에 대 한 공개 토론을 장려, 치료 요법, 잠재적인 합병증, 그리고 지속적인 치료를 위해 필요.
누가 미치지?
발생률과 pemphigus의 보급에 대 한 확실 한 통계는 사용할 수 없습니다., 하지만 새로운 건수의 추정 진단 각 연도 범위에서 높은 5 십만에 당 당 하나의 낮은, pemphigus 유형 및 영향을 받는 인구 민족성에 따라.
인종과 문화적 라인에 걸쳐 사람들에 영향을 미치는 알려진. 그러나, 거기에 특정 그룹의 사람들 (동부 유럽 유태인, 지중해의 사람들, 북부 인도 페르시아 괜찮은) 사람은 질병의 발생률이 높은. 연구 제안 질병 유전.
남자와 여자 똑같이 영향을.
발병 일반적으로 중간 및 오래 된 성인에서 발생 하지만, PV 및 PF에도 젊은 성인과 어린이에 발생.
연구 제안 질병 유전. 몇몇 유전자 표시는 pemphigoid와 연결 하는 동안, 정확한 트리거 또는 트리거 알려져 있지. It is unknown why only some people with these markers develop the disease.
Cats, dogs, horses, and goats can also have PV or PF, although PF is more common.
How do they affect my quality of life?
A diagnosis of pemphigus can be very upsetting. One day you feel perfectly healthy, the next day you learn you have a chronic, life-threatening illness. In general, once pemphigus and pemphigoid are under control, you should be able to return to your normal lifestyle. You may notice there are times when you feel wonderful, and other times when you feel discouraged, in pain, or depressed. There are cases where pemphigus or pemphigoid can be very debilitating, and cause lost time at work, loss of appetite, inability to eat normally, weight loss, loss of sleep, hospitalization, emotional distress, etc. Fortunately, this usually occurs with the onset of the disease, during the search for a correct diagnosis and proper treatment. Once the disease is diagnosed and effective treatment is initiated, you will find your life returning to normal.
In general, pemphigoid can be controlled to the degree that aside from taking some daily medications, the lifestyles of those who live with these diseases are largely unchanged. There are, however, cases where pemphigoid can be very debilitating and cause lost time at work, loss of appetite, inability to eat normally, weight loss, loss of sleep, hospitalization, emotional distress, etc. These effects are most often associated with the onset of the disease during the search for a correct diagnosis. If an early diagnosis is made and treatment initiated, it is very common for the patient to return to a fairly normal lifestyle.</div>
[box type="info"] Much of the impact on lifestyle comes as a result of side effects caused by prednisone.
Much of the impact on lifestyle comes as a result of side effects caused by prednisone.
- Type 2 Diabetes (steroid-induced diabetes) is a common side effect of prednisone and creates a need for a modified diet. Generally, this type of diabetes will diminish as the dosage of prednisone is reduced and goes away when prednisone is stopped.
- Many people on prednisone also experience emotional difficulties and mood changes. If these are continual and severe, other medications are often used to help mitigate these side effects.
- Another commonly reported side effect of prednisone is weight gain. A high protein, low carbohydrate, low fat diet, as well as a regular exercise program is recommended for those taking prednisone.
- Osteoporosis, glaucoma, and cataracts are also known side effects of prednisone, and regular checkups with your health care providers will enable most people on prednisone to effectively counter these side effects with appropriate therapies and attention.
- It is very important to make certain that all physicians, doctors, and specialists involved with a treatment regimen are in contact with one another to avoid conflicting medications and to be sure that each doctor’s treatments are working in harmony with the others. Also, all lab test results should automatically be given to all physicians on a particular case.
Does stress have a negative affect on pemphigus and pemphigoid?
Many people who live with pemphigus report a direct relationship between increased stress and flares in disease activity. Others seem to be unaffected by increased stress.
The possible effect of stress on the onset or subsequent flare of pemphigus or pemphigoid is not clearly understood. Many people who live with P/P report a direct relationship between increased stress and flares in disease activity. Others seem to be unaffected by increased stress.
If you believe stress is related to increased incidence of lesions, it is wise to address and resolve those stress issues. Obviously, many causes of stress are not a matter of choice, but the manner in which an individual deals with the various sources of stress can be modified to minimize the neagative impacts of stress. One of the most common and effective ways to help reduce stress is to openly and honestly discuss it with a spouse, friend, or therapist. Having someone in your corner helps.
Does nutrition play a part in my “new” life?
Many nutrition concerns arise from the medication prednisone. In order to control an outbreak of pemphigus, a prompt response with a large dose of prednisone is generally prescribed.
Use of this drug requires a diet high in protein, low in carbohydrates, low in salt, low in fat, with special attention paid to calcium and potassium levels.
Calcium with vitamin D supplements are routinely ordered.
Acidophilus, a beneficial bacteria found in yogurt, as well as in dietary supplements, is recommended for those who frequently use antibiotics. It also may helps prevent yeast infections.
It has been documented that some some patients have a sensitivity to garlic, onions and leeks (the Alium food group). These foods and possibly others may trigger or worsen flair-ups.
If you suspect that eating certain foods causes blisters, 그런 다음 두 번째 시간 시도, 그리고 다시 발생 하는 경우, 귀하의 식단에서 음식을 제거합니다. 현재 귀하의 다이어트를 논의 해야 합니다., 약물, 귀하의 의사 또는 영양사 귀하의 다이어트를 변경 하기 전에 라이프 스타일.
- 아무도 이러한 식품을 피해 야 한다, 그러나 배려는 개별적으로 주어질 수 있다, 일부 환자 보고 특정 식품 그룹 4 개의 성분 중 하나 이상을 포함 하 여 질병의 격화: thiol, isothiocyanates, 페 놀 또는 tannins.
- Thiols: 부추속의 다른 구성원과 마늘 양파와 같은 식물을 포함 하는 그룹, 샬 롯, 부추 및 골 파.
- Isothiocyanates (겨자 기름): 발견 3200 겨자를 포함 한 식물의 종, 고추 냉이, 겨울 유채과 야채, 순 무, 브로콜리, 무, 양배추, brussel 콩나물과 콜리플라워. 케이 퍼. 겨자 합성 오일.
- 페 놀: Urushiol 접촉 피부염을 일으킬 수 있고 가장 주목할 만한에 포이즌 아이비, 옻과 망고과 관련 있는 독이 옻나무, 피스타치오와 캐슈. 인공 감미료 아스파탐은 페 놀과 공통점이 많은 식품 첨가물. 페 놀 계 및 cinnamic 산에, 그리고 pinene. 그것은 토마토에, 감자, 망고 및 바나나와 우유와 우유 제품 때 소 소비 페 놀 생산 엮 여 먹이 면 실 등.
- Tannins: 탄 닌의 일반적인 소스는 콜라 너트, 차, 커피, 라즈베리, 체리, 적갈색, 블랙베리, 아보카도, 바나나, 애플, 망고, 배, 가지와 포도 스킨, 커피와 코코아 씨앗, 생강, 인삼, 마늘, 로즈마리, 칡,.
