Materiały źródłowe

• #1 Pemphigus – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus

  Pemphigus is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. In pemphigus, autoantibodies form against desmoglein, which forms the „glue” that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes detached, a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a large area of the skin. […] The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus) vary in severity. Skin lesions caused by pemphigus can lead to fatal infections, so treatment is extremely important.

• #1 Pemphigus: causes, symptoms, and treatment here – TeleMed2U

  https://www.telemed2u.com/dermatology/pemphigus

  Pemphigus can develop slowly, or appear suddenly and spread quickly. An otherwise healthy person can quickly become very sick, with pain and extreme fatigue. Widespread blisters can be life threatening. […] Pemphigus symptoms depend on the type you have. Many cases can cause these symptoms: Blisters on skin and mucous membranes that are soft and limp. Blisters begin in one area, on both normal-looking skin or inflamed skin. Blisters break open easily, ooze fluid and become open sores; sores can crust over. […] Sores can develop in the mouth, eyes, nose, genitals, anus, throat or esophagus. Sores can become widespread, infected and painful. […] Sores in the mouth or throat can cause hoarseness, make talking difficult, and cause painful eating and drinking. […] Sores heal very slowly or may never heal, leaving blisters in the same area for many years.

• #1 What is Pemphigus? Symptoms & Causes | NIAMS

  https://www.niams.nih.gov/health-topics/pemphigus

  Pemphigus is a disease that causes blistering of the skin and the inside of the mouth, nose, throat, eyes, and genitals. […] The main symptom of pemphigus is blistering of the skin and in some cases, the mucosal surfaces, such as the inside of the mouth, nose, throat, eyes, and genitals. The blisters are fragile and tend to burst, causing crusty sores. Blisters on skin may join together, forming raw-looking areas that are prone to infection and that ooze large amounts of fluid. The symptoms can vary depending on the type of pemphigus. […] Pemphigus vulgaris blisters often start in the mouth but can develop on the skin later on. The skin may become so fragile that it peels off by rubbing a finger on it. Mucosal surfaces such as those of the nose, throat, eyes, and genitals may also be affected. Blisters form within the deep layer of the epidermis, and they are often painful.

• #1 Pemphigus: Types, Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/21130-pemphigus

  Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. These often break open, causing pain and leaving you vulnerable to infection. Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease. The blisters are soft and break open easily to form painful sores. Without treatment, they can spread over large areas of your body and have a risk of infection. Pemphigus causes blisters and sores to form on your skin. These lesions form quickly and can last for years, with new blisters appearing in the same area of your skin after one blister goes away. These lesions can be painful and cause additional symptoms like infections. Contact your healthcare provider if you have blisters that are widespread across your body, as they could be life-threatening. Symptoms vary based on the type of pemphigus you have but could include: Fluid-filled bump or bubble on your skin (blister). The skin around the blister is pink to red. Sores that have a crusty appearance. Blisters or sores leak clear fluid or bleed lightly. Your skin around the blisters is fragile and peels in layers or scales. Pain on or near your affected skin. Itchy skin. Blisters and sores can easily become infected. Skin symptoms of an infection include: White or yellow pus fills the blister and leaks out if the blister breaks open. Pain or a burning sensation to the touch. Yellow crust forms on the blister if it breaks open. Skin doesn’t heal. The area on or around the blister swells or gets bigger. Severe symptoms of pemphigus include: Fever. Fatigue. Muscle weakness. Vision problems and light sensitivity. If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms. Treatment for pemphigus takes time before you see results. With treatment, you’ll notice new blisters stop forming after several weeks and your skin will begin to heal. It could take months for your blisters and sores to heal completely.

• #1 Pemphigus: Signs and symptoms

  https://www.aad.org/public/diseases/a-z/pemphigus-symptoms

  When someone develops pemphigus, it can develop slowly, causing blisters in the same area for years. […] Signs and symptoms can also show up quickly. Blisters can appear suddenly and spread. Widespread pemphigus can be life threatening. It can turn an otherwise healthy person into one who is extremely sick, incredibly tired, and in pain. […] The sores are often painful, but rarely itchy. Some people say the affected skin burns. […] The sores tend to heal slowly, and some never heal. When a sore heals, you may see a dark spot in its place. This is not a scar. Some people see dark spots when their skin heals. The dark spots often fade on their own, but this can take time. […] Painful mouth sores are common in people who have pemphigus vulgaris, the most common type of pemphigus. About 50% to 70% of people who have pemphigus vulgaris develop mouth sores before blisters appear on their skin.

• #1 Pemphigus – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pemphigus/symptoms-causes/syc-20350404

  Pemphigus is a rare skin disorder with blistering of the skin and mucous membranes. The most common type is pemphigus vulgaris, which involves painful sores and blisters on the skin and in the mouth. […] Pemphigus causes blisters on the skin and mucous membranes. The blisters break easily, leaving open sores. The sores may become infected and ooze. […] The symptoms of two common types of pemphigus are as follows: Pemphigus vulgaris. This type usually begins with blisters in the mouth and then on the skin or genital mucous membranes. They’re often painful but don’t itch. Blisters in the mouth or throat may make it hard to talk, drink and eat. […] Pemphigus is easier to control if caught and treated early. It’s usually treated with medicines that you take long term. The sores may heal slowly or not at all. The condition can become life-threatening if the sores become infected.

• #1 Pemphigus – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus

  Pemphigus vulgaris (PV – ICD-10 L10.0) is the most common form of the disorder and occurs when antibodies attack desmoglein 3. Sores often originate in the mouth, making eating difficult and uncomfortable. […] If not treated, pemphigus can be fatal, usually from overwhelming opportunistic infection of lesions.

• #1 P/P Clinical Information – IPPF

  https://www.pemphigus.org/p-p-clinical-information/

  Pemphigus is defined as a group of life-threatening blistering disorders characterized by acantholysis resulting in the formation of intraepithelial blisters in mucous membranes and skin. Patients with pemphigus develop mucosal erosions and/or flaccid bullae (blisters), erosions, or pustules on skin (small bumps that fill with pus or fluid). […] The four major types of pemphigus include pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus. […] Pemphigus vulgaris generally is more severe than pemphigus foliaceus. Pemphigus vulgaris usually presents with widespread mucocutaneous blisters and erosions. […] Almost all patients with pemphigus vulgaris develop mucosal involvement. The oral cavity is the most common site of mucosal lesions and often represents the initial site of disease.

• #1 Pemphigus vulgaris

  https://www.nhs.uk/conditions/pemphigus-vulgaris/

  Pemphigus vulgaris causes blisters that burst easily and leave very sore patches. […] The most common area affected is inside the mouth and throat, which can make swallowing painful. […] Many people also get blisters on their skin. These usually appear a few months after the mouth is affected. […] The blisters and sore areas can cover a large area of the body. Sometimes they can also affect the eyes, genitals or anus. […] Blisters caused by pemphigus vulgaris usually come and go over time. […] It usually takes a few weeks for the blisters to heal. Once this happens, you’ll probably need treatment for several years to help stop the blisters coming back. […] Pemphigus vulgaris can lead to other problems, such as: blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis. […] weight loss and malnutrition because of pain when eating and drinking. […] pain during sex and pain when peeing or pooing (if your genitals or anus are affected).

• #1 Pemphigus Vulgaris: A Complete Overview — DermNet

  https://dermnetnz.org/topics/pemphigus-vulgaris

  Most patients with pemphigus vulgaris first present with lesions on the mucous membranes such as the mouth and genitals. Blisters usually develop on the skin after a few weeks or months, although in some cases, mucosal lesions may be the only manifestation of the disease. […] Skin lesions appear as thin-walled flaccid blisters filled with clear fluid that easily rupture causing itchy and painful erosions. They most often arise on the upper chest, back, scalp, and face. Erosions in the skin folds may develop into vegetative lesions which are granular and crusted (pemphigus vegetans). The skin around the nails may be painful, red, and swollen. […] The inside of the mouth is commonly involved in pemphigus vulgaris. Involvement of the pharynx and larynx cause pain on swallowing and a hoarse voice. Nasal involvement causes congestion and bleeding. The conjunctiva, oesophagus, labia, vagina, cervix, penis, urethra and anus may also be affected.

• #1 Pemphigus: Signs and symptoms

  https://www.aad.org/public/diseases/a-z/pemphigus-symptoms

  These sores can be so painful that some people stop eating solid food and use a straw to drink. If sores develop in the throat, talking can be painful. […] Most people who have pemphigus vulgaris develop mouth sores at some point. […] Painful sores can develop in the tissue lining the inside of the eyes and nose, genitals, anus, and other areas of the body. Even the esophagus (the tube that connects the throat to the stomach) can develop blisters and sores in rare cases. […] Other signs and symptoms of pemphigus include pain, fatigue, weakness, light sensitivity, and eye problems.

• #1 Pemphigus Foliaceus: Causes, Symptoms and Images — DermNet

  https://dermnetnz.org/topics/pemphigus-foliaceus

  Pemphigus foliaceus is characterised by blistering lesions on otherwise healthy-looking skin. Blisters tend to form when the skin is rubbed (Nikolsky sign). Pemphigus foliaceus produces superficial blisters confined to the skin, without involvement of mucous membranes. The patient with pemphigus foliaceus is usually otherwise in good health. Small fluid-filled blisters first form on the trunk. Being superficial within the upper epidermis, they rupture very easily, and only erosions may be seen. On the face, scalp and upper trunk, the lesions are often scaly and crusty on a red and inflamed base. A burning sensation or localised pain may be felt. […] Spontaneous remission may occur in some patients while in others, the problem may persist for several years.

• #1 Pemphigus Foliaceus: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23029-pemphigus-foliaceus

  Pemphigus foliaceus is an autoimmune condition that causes painful and itchy blisters and sores on your skin, most often on your face, scalp and trunk. Symptoms can reduce with topical creams, ointments or drugs. This condition isnt contagious. […] Symptoms of pemphigus foliaceus arise on average between ages 50 and 60. The condition can affect any age, including children. […] Pemphigus foliaceus causes painful blisters to form on your skin. The blisters are fragile and break open easily, which makes them turn into sores. When the blisters break, a scaly, crusty exterior forms on the affected area of your skin. These can be itchy and cause discomfort. […] Symptoms of pemphigus foliaceus can range in severity and include: Blisters or small, fluid-filled, raised, red bumps on the top layer of your skin (epidermis) that can cover a large area of your body. Blisters are soft and break open easily. Broken blisters turn into sores that have a scaly, crusty texture. Blisters and sores cause itchiness, pain or a burning sensation.

• #1 P/P Clinical Information – IPPF

  https://www.pemphigus.org/p-p-clinical-information/

  Since mucosal blisters erode quickly, erosions are often the only clinical findings. […] Most patients also develop cutaneous involvement manifesting as flaccid blisters on normal-appearing or erythematous skin. The blisters rupture easily, resulting in painful erosions that bleed easily. […] Pemphigus foliaceus usually develops in a seborrheic distribution. The scalp, face, and trunk are common sites of involvement. The skin lesions usually consist of small, scattered superficial blisters that rapidly evolve into scaly, crusted erosions. […] Pemphigus foliaceus progresses to involve the entire skin surface as an exfoliative erythroderma. […] Pemphigus vulgaris (the most common form of pemphigus) occurs worldwide and the frequency is influenced by geographic location and ethnicity. […] Pemphigus usually occurs in adults, with an average age of onset between 40 to 60 years of age for pemphigus vulgaris and nonendemic pemphigus foliaceus.

• #1 Pemphigus Erythematosus: Practice Essentials, Etiology, Epidemiology

  https://emedicine.medscape.com/article/1063881-overview

  Patients with pemphigus erythematosus present with vesiculobullae or superficially eroded lesions, which may ooze and crust, particularly in sun-exposed areas, such as the face, the upper part of the chest, and the back. […] Onset and progression of pemphigus erythematosus are typically slow. […] Pemphigus erythematosus lesions typically involve the scalp, the face, the upper part of the chest, and the back. […] Patients with classic pemphigus erythematosus present with small, flaccid bullae with scaling and crusting. […] With extensive involvement, pemphigus erythematosus patients may present with an exfoliative erythroderma. […] Patients with pemphigus erythematosus do not typically develop mucous membrane involvement. […] The prognosis of pemphigus erythematosus is better than that of pemphigus vulgaris. With good dermatologic care, patients with pemphigus erythematosus are often able to live normal lives. Some patients may ultimately develop symptoms classified as criteria for systemic lupus erythematosus (SLE) by the American College of Rheumatology (ACR).

• #1 Pemphigus – IPPF

  https://www.pemphigus.org/pemphigus/

  Blistering may be accompanied by severe pain, itching, burning, and stinging. If extensive, blistering can lead to life-threatening fluid loss, infection, and disfigurement. […] Pemphigus foliaceous usually affects the scalp, face, toros, and/or armpits. The skin lesions usually are small, scattered blisters that rapidly evolve into scaly, crusted erosions. […] Pemphigus foliaceous is superficial with skin lesions. The mucous membranes are typically not affected. […] Paraneoplastic pemphigus is an autoimmune multi-organ syndrome associated with neoplastic disease. Typically, patients suffer from severe and critical mucosal involvement with extensive, stubborn inflammation of the mucous linings. The skin indicators vary, and include blisters, erosions, and lichen-type lesions that may resemble other autoimmune blistering diseases, erythema multiforme, graft versus host disease, or lichen planus.

• #1 Paraneoplastic pemphigus: Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/paraneoplastic-pemphigus

  The outlook for those with paraneoplastic pemphigus can depend on the cause and what complications develop. The mortality rate is generally high at around 70% to 90%, due to factors such as the underlying cancer, severe infections, and bronchiolitis obliterans. […] Paraneoplastic pemphigus can lead to life threatening complications such as infections and bronchiolitis obliterans.

• #1 Pemphigus: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pemphigus

  Lesions may extend to cause hoarseness. […] Eating and drinking may become very uncomfortable. […] Other mucous membranes (conjunctivae, oesophagus and genitalia) may be involved. […] Specific features […] Pemphigus vulgaris (PV): […] Most commonly presents with painful erosions or blisters on the oral mucosa, and these oral lesions may occur up to four months before skin lesions become evident. […] Occasionally, the lesions will remain confined to the mouth. […] A small number of patients will present with cutaneous blistering first; however, all will go on to develop oral lesions. […] Pemphigus foliaceus (PF): […] Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. […] […] […] Prognosis […] Untreated, the mortality associated with pemphigus vulgaris (PV) was 75%. The use of corticosteroids and adjuvant drugs has reduced the mortality rate significantly. It has been reported as 12% in the UK, with a 3 x higher risk of death compared with age-matched controls. […] Many experience serious side-effects from the drugs used, and many of the deaths occurring today are as a result of infection due to the immunosuppressive effects of the treatment. The outlook is worst in elderly patients and patients with extensive disease.

• #1 Pemphigus Vulgaris: A Complete Overview — DermNet

  https://dermnetnz.org/topics/pemphigus-vulgaris

  Features of oral mucosal pemphigus include: Oral lesions in 50-70% of patients, Superficial blistering and erosions, Widespread involvement within the mouth, Painful, slow-to-heal ulcers, Spread to the larynx causing hoarseness when talking, Difficulty eating and drinking. […] Pemphigus vulgaris can cause very extensive, life-threatening erosions, especially if the diagnosis is delayed. Other potentially severe complications may include: Secondary bacterial infection, Fungal infection, especially candida, Viral infection, especially herpes simplex, Nutritional deficiencies due to difficulty eating, Complications of systemic steroids especially infections and osteoporosis, Complications of immune suppressive treatments, The psychological effects of severe skin disease and its treatment (anxiety and depression), An elevated risk of cardiovascular disease (atherosclerosis, heart failure, arrhythmia, thromboembolism, and cardiovascular death).

• #1 Pemphigus vulgaris – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/pemphigus-vulgaris/

  Pemphigus vulgaris may cause severe blistering of the skin and the mucous membranes lining the mouth, nose, throat, eyes and genital area. Blisters develop in the upper layer of the skin and have a thin and fragile outer surface that breaks away easily leaving raw areas (erosions) that can be extensive and painful. […] Skin erosions (sores) are painful and can affect quality of life including disturbing sleep. Mouth and throat lesions can interfere with eating and drinking and so cause weight loss. Pemphigus vulgaris can also affect other mucous membranes such as the genital area, leading to painful sexual intercourse, urination and bowel movements, the nose causing stuffiness and blood-stained crusts; and the conjunctiva of the eyes causing sore, red eyes (requiring assessment by an ophthalmologist).

• #1 Pemphigus Foliaceus: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23029-pemphigus-foliaceus

  The most common places on your body where youll find blisters and sores from a pemphigus foliaceus diagnosis are your: Scalp. Face. Neck. Back. Chest. […] Symptoms could flare or arise again in the future since pemphigus is a chronic condition. […] There is no cure for pemphigus foliaceus. Symptoms of pemphigus foliaceus dont interfere with your life expectancy. The condition can cause painful and itchy blisters and sores on your skin that heal with treatment. Blisters rarely cause scars. An injury or frequent scratching of a blister or a sore could lead to scarring.

• #1 Pemphigus Foliaceus: Symptoms, Causes, Treatment & More

  https://www.healthline.com/health/pemphigus-foliaceus

  Pemphigus foliaceus causes fluid-filled blisters to form on your skin, often on your chest, back, and shoulders. At first the blisters are small, but they gradually grow and increase in number. Eventually they can cover your whole torso, face, and scalp. […] The blisters break open easily. Fluid may ooze from them. If you rub your skin, the whole top layer can separate from the bottom later and peel off in a sheet. […] After the blisters break open, they can form sores. The sores scale and crust over. […] Although pemphigus foliaceus usually isnt painful, you may feel pain or a burning sensation in the area of the blisters. The blisters may also itch. […] Some people get better without treatment. Others may live with the disease for many years. You might need to take medicine for years to prevent the blisters from coming back.

• #1 Pemphigus Vulgaris – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. […] Flaccid bullae, which are the primary lesions of pemphigus vulgaris, cause widespread and painful skin, oral, and other mucosal erosions. About half of patients have only oral erosions, which rupture and remain as chronic, painful lesions for variable periods. Often, oral lesions precede skin involvement. Dysphagia and poor oral intake are common because lesions also may occur in the upper esophagus. Cutaneous bullae typically arise in normal-appearing skin, rupture, and leave a raw area with crusting. Itching is usually absent. Erosions often become infected. If large portions of the body are affected, fluid and electrolyte loss may be significant. […] Without treatment, pemphigus vulgaris is often fatal, usually within 5 years of disease onset. Systemic corticosteroid and immunosuppressive therapy has improved prognosis, but death may still result from complications of therapy.

• #1 Pemphigus Vulgaris – Skin Disorders – MSD Manual Consumer Version

  https://www.msdmanuals.com/home/skin-disorders/blistering-diseases/pemphigus-vulgaris

  Pemphigus vulgaris is a rare, severe autoimmune disorder in which blisters of varying sizes break out on the skin and on the lining of the mouth and other mucous membranes. […] The major symptom of pemphigus vulgaris is the development of clear, soft, and painful (sometimes tender) blisters of various sizes. In addition, the top layer of skin may detach from the lower layers in response to slight pinching or rubbing, causing it to peel off in sheets and to leave painful areas of open skin (erosions). […] The blisters often first appear in the mouth and soon rupture, forming painful sores (ulcers). More blisters and ulcers may follow until the entire lining of the mouth is affected, causing difficulty swallowing, eating, and drinking. Blisters form in the throat as well. […] Blisters can form on the skin and rupture, leaving raw, painful, crusted wounds. The person feels generally ill. Blisters may be widespread and, once ruptured, they may become infected. When severe, pemphigus vulgaris is as harmful as a serious burn. Similar to a burn, the damaged skin oozes large amounts of fluid and is prone to infection by many types of bacteria.

• #1 Pemphigus vulgaris – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/pemphigus-vulgaris/

  The most common area to be affected is the inside of the mouth. This is most commonly the first area to be affected, and skin lesions often appear later. Other areas, as described above, are less commonly affected. […] Pemphigus vulgaris is a long-lasting (chronic) disease and will not usually settle without treatment. There will be occasions when it flares up and other occasions when it improves. Currently, there is no way of predicting when flares will occur or how severe they will be. Treatment is aimed at controlling the disease and preventing flares rather than a cure. […] Treatment is important because severe pemphigus vulgaris is a serious and potentially life-threatening condition if not treated early and effectively. Treatment involves long-term use of oral medicines. In severe cases, intravenous medication may be needed as a hospital in-patient. Most treatments work by suppressing the immune system.

• #1 Pemphigus vulgaris – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus_vulgaris

  Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. […] Over time the condition inevitably progresses without treatment: lesions increase in size and distribution throughout the body, behaving physiologically like a severe burn. […] Pemphigus vulgaris most commonly presents with oral blisters (buccal and palatine mucosa, especially), but also includes cutaneous blisters. […] Blisters commonly erode and leave ulcerated lesions and erosions. […] Severe pain with chewing can lead to weight loss and malnutrition. […] The disease arises most often in middle-aged or older people, usually starting with a blister that ruptures easily. […] The lesions can become quite extensive. […] If left untreated, 8 of 10 people with the disease die within a year with a cause of death being infection or loss of fluids, which is very common for raw, open sores that are characteristic of P. vulgaris.

• #1

  https://111.wales.nhs.uk/pemphigusvulgaris/

  Pemphigus vulgaris can lead to other problems, such as blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis. […] You’ll have regular check-ups to look for these problems and may be referred to other specialists (such as a dietitian) if needed. […] The main aim of treatment is to heal the blisters and prevent new ones forming. […] You usually start on a high dose to get your symptoms under control. This can lead to a noticeable improvement within a few days, although it usually takes 2 to 3 weeks to stop new blisters forming and 6 to 8 weeks for existing blisters to heal. […] It can take a while to find the best dose for you. It may take a few months to reach a balance between controlling your symptoms and limiting unpleasant side effects.

• #1 Pemphigus vulgaris – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/pemphigus-vulgaris/

  The aims of treatment are to prevent new blisters forming and to heal broken areas of skin. It usually takes about 2 to 3 weeks to stop new blisters developing, and 6 to 8 weeks for healing to occur. Once the disease is controlled, the dose of the medication can be reduced slowly to the lowest effective level.

• #1 Pemphigus and Pemphigoid > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/pemphigus-and-pemphigoid

  Symptoms include painful blisters in the mouth, on the skin, or elsewhere in the body. […] Symptoms of these diseases can include pain in the mouth, bleeding when brushing teeth, recurrent bloody noses, red eyes, pain while urinating or with bowel movements, or tenderness during intercourse, depending on where the blisters develop. Bullous pemphigoid is often very itchy. Some people will have visible sores on their skin either fluid-filled blisters or open sores, says Dr. Tomayko. Some people have blisters only in their mouths or in another mucous membrane, like the eyes. Others have them only on their skin, and some have a combination of these. […] Pemphigus is characterized by shallow ulcers or fragile blisters that break open quickly. Pemphigoid presents with stronger or tense blisters that don’t open easily. Those with pemphigoid are also more likely to have hot, red and itchy hive spots.

• #1 Pemphigus and Pemphigoid > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/pemphigus-and-pemphigoid

  Because the blisters are tense, those resulting from pemphigoid are more likely to leave permanent marks on the skin, Dr. Tomayko says. When the eyes are affected, scarring and vision loss can result. It is important to seek care. […] The majority of patients with these disorders will see very significant improvement after three months of treatment. Some patients go into a long-term remission after that, while others will need a second treatment a year later. Most will require ongoing treatment. […] Even without any treatment, these disorders tend to burn out after five to 10 years in some people. […] Patients are not cured, per se, but the symptoms can be controlled, skin will heal if cared for properly, and, over the long term, there is a good likelihood that the disorders will go away.

• #1 What’s Pemphigus? What Causes It?

  https://www.webmd.com/skin-problems-and-treatments/what-is-pemphigus

  If you have it, you may have blisters in your mouth that you cant treat. Treating the cancer will help to ease the blisters. […] If pemphigus isnt treated, it could be life-threatening. Sometimes, you may have to be admitted to the hospital until you get better. […] At least 75% of people with pemphigus will have a complete remission, or no evidence of the disease, after 10 years of treatment. Some people must take medications for the rest of their lives to keep pemphigus symptoms from coming back.

• #1 Pemphigus Vulgaris: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1064187-overview

  The severity and natural history of pemphigus vulgaris are variable. Before the advent of steroids, most patients with pemphigus vulgaris died. Treatment with systemic steroids has reduced the mortality dramatically, to approximately 5-15%. If not properly treated, pemphigus vulgaris still is often fatal because of the susceptibility to infection and fluid and electrolyte disturbances. […] Mortality in patients with pemphigus vulgaris is three times higher than that in the general population. Most deaths occur during the first few years of disease; if the patient survives 5 years, the prognosis is good. Early disease probably is easier to control than widespread disease, and mortality may be higher if therapy is delayed. Complications secondary to the use of high-dose corticosteroids contribute to mortality as well. […] Relapses may occur in more than 50% of patients with pemphigus (including variants other than pemphigus vulgaris).

• #1 Pemphigus: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/pemphigus/diagnosis-treatment-and-steps-to-take

  Early diagnosis is important, so if you have blisters on the skin or in the mouth that do not go away, it is important to see a doctor as soon as you can. […] Symptoms of pemphigus may go away after many years of treatment, but most people need to continue taking medications to keep the disease under control. […] In some cases, a person with pemphigus may need to be hospitalized to treat health problems that the disease or its treatment can cause. Widespread sores on the skin can result in dehydration or infection, and painful blisters in the mouth can make it difficult to eat. […] Pemphigus and its treatments can be debilitating and cause lost time at work, weight loss, sleep problems, and emotional distress.

• #2 P/P Clinical Information – IPPF

  https://www.pemphigus.org/p-p-clinical-information/

  Pemphigus is defined as a group of life-threatening blistering disorders characterized by acantholysis resulting in the formation of intraepithelial blisters in mucous membranes and skin. Patients with pemphigus develop mucosal erosions and/or flaccid bullae (blisters), erosions, or pustules on skin (small bumps that fill with pus or fluid). […] The four major types of pemphigus include pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus. […] Pemphigus vulgaris generally is more severe than pemphigus foliaceus. Pemphigus vulgaris usually presents with widespread mucocutaneous blisters and erosions. […] Almost all patients with pemphigus vulgaris develop mucosal involvement. The oral cavity is the most common site of mucosal lesions and often represents the initial site of disease.

• #2 Pemphigus: Signs and symptoms

  https://www.aad.org/public/diseases/a-z/pemphigus-symptoms

  When someone develops pemphigus, it can develop slowly, causing blisters in the same area for years. […] Signs and symptoms can also show up quickly. Blisters can appear suddenly and spread. Widespread pemphigus can be life threatening. It can turn an otherwise healthy person into one who is extremely sick, incredibly tired, and in pain. […] The sores are often painful, but rarely itchy. Some people say the affected skin burns. […] The sores tend to heal slowly, and some never heal. When a sore heals, you may see a dark spot in its place. This is not a scar. Some people see dark spots when their skin heals. The dark spots often fade on their own, but this can take time. […] Painful mouth sores are common in people who have pemphigus vulgaris, the most common type of pemphigus. About 50% to 70% of people who have pemphigus vulgaris develop mouth sores before blisters appear on their skin.

• #2 Pemphigus: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/pemphigus-treatment

  If you are diagnosed with pemphigus, you’ll likely need treatment. Without treatment, pemphigus tends to worsen. In some people, pemphigus can become life-threatening without treatment. […] Pemphigus cannot be cured, but with treatment, most people can control their pemphigus. […] Treatment can reduce (and sometimes clear) the blisters and sores caused by all types of pemphigus. Treatment can also prevent pemphigus from worsening. […] Before medicines like prednisone and azathioprine were used to treat pemphigus, a person lived about 5 years after getting pemphigus vulgaris, the most common type. Thanks to medicines and other treatments, this has changed. Few people die of pemphigus. […] Serious weight loss is another problem that pemphigus can cause.

• #2 P/P Clinical Information – IPPF

  https://www.pemphigus.org/p-p-clinical-information/

  Since mucosal blisters erode quickly, erosions are often the only clinical findings. […] Most patients also develop cutaneous involvement manifesting as flaccid blisters on normal-appearing or erythematous skin. The blisters rupture easily, resulting in painful erosions that bleed easily. […] Pemphigus foliaceus usually develops in a seborrheic distribution. The scalp, face, and trunk are common sites of involvement. The skin lesions usually consist of small, scattered superficial blisters that rapidly evolve into scaly, crusted erosions. […] Pemphigus foliaceus progresses to involve the entire skin surface as an exfoliative erythroderma. […] Pemphigus vulgaris (the most common form of pemphigus) occurs worldwide and the frequency is influenced by geographic location and ethnicity. […] Pemphigus usually occurs in adults, with an average age of onset between 40 to 60 years of age for pemphigus vulgaris and nonendemic pemphigus foliaceus.

• #2 Pemphigus Vulgaris: A Complete Overview — DermNet

  https://dermnetnz.org/topics/pemphigus-vulgaris

  Most patients with pemphigus vulgaris first present with lesions on the mucous membranes such as the mouth and genitals. Blisters usually develop on the skin after a few weeks or months, although in some cases, mucosal lesions may be the only manifestation of the disease. […] Skin lesions appear as thin-walled flaccid blisters filled with clear fluid that easily rupture causing itchy and painful erosions. They most often arise on the upper chest, back, scalp, and face. Erosions in the skin folds may develop into vegetative lesions which are granular and crusted (pemphigus vegetans). The skin around the nails may be painful, red, and swollen. […] The inside of the mouth is commonly involved in pemphigus vulgaris. Involvement of the pharynx and larynx cause pain on swallowing and a hoarse voice. Nasal involvement causes congestion and bleeding. The conjunctiva, oesophagus, labia, vagina, cervix, penis, urethra and anus may also be affected.

• #2 Pemphigus: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pemphigus

  Lesions may extend to cause hoarseness. […] Eating and drinking may become very uncomfortable. […] Other mucous membranes (conjunctivae, oesophagus and genitalia) may be involved. […] Specific features […] Pemphigus vulgaris (PV): […] Most commonly presents with painful erosions or blisters on the oral mucosa, and these oral lesions may occur up to four months before skin lesions become evident. […] Occasionally, the lesions will remain confined to the mouth. […] A small number of patients will present with cutaneous blistering first; however, all will go on to develop oral lesions. […] Pemphigus foliaceus (PF): […] Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. […] […] […] Prognosis […] Untreated, the mortality associated with pemphigus vulgaris (PV) was 75%. The use of corticosteroids and adjuvant drugs has reduced the mortality rate significantly. It has been reported as 12% in the UK, with a 3 x higher risk of death compared with age-matched controls. […] Many experience serious side-effects from the drugs used, and many of the deaths occurring today are as a result of infection due to the immunosuppressive effects of the treatment. The outlook is worst in elderly patients and patients with extensive disease.

• #2 Pemphigus Erythematosus: Practice Essentials, Etiology, Epidemiology

  https://emedicine.medscape.com/article/1063881-overview

  Patients with pemphigus erythematosus present with vesiculobullae or superficially eroded lesions, which may ooze and crust, particularly in sun-exposed areas, such as the face, the upper part of the chest, and the back. […] Onset and progression of pemphigus erythematosus are typically slow. […] Pemphigus erythematosus lesions typically involve the scalp, the face, the upper part of the chest, and the back. […] Patients with classic pemphigus erythematosus present with small, flaccid bullae with scaling and crusting. […] With extensive involvement, pemphigus erythematosus patients may present with an exfoliative erythroderma. […] Patients with pemphigus erythematosus do not typically develop mucous membrane involvement. […] The prognosis of pemphigus erythematosus is better than that of pemphigus vulgaris. With good dermatologic care, patients with pemphigus erythematosus are often able to live normal lives. Some patients may ultimately develop symptoms classified as criteria for systemic lupus erythematosus (SLE) by the American College of Rheumatology (ACR).

• #2 Pemphigus Foliaceus: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23029-pemphigus-foliaceus

  The most common places on your body where youll find blisters and sores from a pemphigus foliaceus diagnosis are your: Scalp. Face. Neck. Back. Chest. […] Symptoms could flare or arise again in the future since pemphigus is a chronic condition. […] There is no cure for pemphigus foliaceus. Symptoms of pemphigus foliaceus dont interfere with your life expectancy. The condition can cause painful and itchy blisters and sores on your skin that heal with treatment. Blisters rarely cause scars. An injury or frequent scratching of a blister or a sore could lead to scarring.

• #2 Paraneoplastic pemphigus: Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/paraneoplastic-pemphigus

  Paraneoplastic pemphigus is a rare autoimmune skin condition that causes blisters and oral lesions. It usually develops due to underlying cancer. Treatment is essential for managing symptoms and improving outlook. […] Paraneoplastic pemphigus causes oral symptoms, including inflammation, which may be the only symptom in some cases, erosions on the mucous membranes, blisters filled with fluid or blood, hemorrhagic crusting, and ulcers. […] A person may also experience skin symptoms such as very itchy widespread skin lesions, which may look red or discolored, bumps or papules on the skin, blisters, scaly plaques, and erythroderma, which is widespread redness or skin discoloration. […] Paraneoplastic pemphigus can cause numerous complications, such as a loss of fluid and protein from skin erosions, electrolyte imbalance or loss of electrolytes, infection and sepsis due to loss of skin barrier function, malnutrition and dehydration due to painful lesions in the mouth, lesions that are resistant to treatment, and eye complications.

• #2 Pemphigus vulgaris

  https://www.nhs.uk/conditions/pemphigus-vulgaris/

  Pemphigus vulgaris causes blisters that burst easily and leave very sore patches. […] The most common area affected is inside the mouth and throat, which can make swallowing painful. […] Many people also get blisters on their skin. These usually appear a few months after the mouth is affected. […] The blisters and sore areas can cover a large area of the body. Sometimes they can also affect the eyes, genitals or anus. […] Blisters caused by pemphigus vulgaris usually come and go over time. […] It usually takes a few weeks for the blisters to heal. Once this happens, you’ll probably need treatment for several years to help stop the blisters coming back. […] Pemphigus vulgaris can lead to other problems, such as: blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis. […] weight loss and malnutrition because of pain when eating and drinking. […] pain during sex and pain when peeing or pooing (if your genitals or anus are affected).

• #2 Pemphigus Vulgaris: A Complete Overview — DermNet

  https://dermnetnz.org/topics/pemphigus-vulgaris

  Features of oral mucosal pemphigus include: Oral lesions in 50-70% of patients, Superficial blistering and erosions, Widespread involvement within the mouth, Painful, slow-to-heal ulcers, Spread to the larynx causing hoarseness when talking, Difficulty eating and drinking. […] Pemphigus vulgaris can cause very extensive, life-threatening erosions, especially if the diagnosis is delayed. Other potentially severe complications may include: Secondary bacterial infection, Fungal infection, especially candida, Viral infection, especially herpes simplex, Nutritional deficiencies due to difficulty eating, Complications of systemic steroids especially infections and osteoporosis, Complications of immune suppressive treatments, The psychological effects of severe skin disease and its treatment (anxiety and depression), An elevated risk of cardiovascular disease (atherosclerosis, heart failure, arrhythmia, thromboembolism, and cardiovascular death).

• #2 Pemphigus Vulgaris – Sussex Community Dermatology Service

  https://sussexcds.co.uk/patient-information/pemphigus-vulgaris/

  In most patients, the blisters and erosions start first in the mouth, and appear later on the skin. In a few, the skin is affected first. Most patients get erosions in their mouth at some time; but some never get blisters or erosions on their skin. […] No, it will not clear without treatment. There will be times when it flares up, and others when it gets better. There is no way of knowing when these flare-ups will happen or how bad they will be. However, pemphigus vulgaris can be controlled by long-term treatment which may eventually result in no further flare ups. […] The initial aim of treatment is to prevent new blisters forming and to heal the existing ones this is disease control. It usually takes about 2 to 3 weeks to stop new blisters coming up, and sometimes as long as 6 to 8 weeks for healing to be complete.

• #2 What is Pemphigus? Symptoms & Causes | NIAMS

  https://www.niams.nih.gov/health-topics/pemphigus

  Pemphigus is a disease that causes blistering of the skin and the inside of the mouth, nose, throat, eyes, and genitals. […] The main symptom of pemphigus is blistering of the skin and in some cases, the mucosal surfaces, such as the inside of the mouth, nose, throat, eyes, and genitals. The blisters are fragile and tend to burst, causing crusty sores. Blisters on skin may join together, forming raw-looking areas that are prone to infection and that ooze large amounts of fluid. The symptoms can vary depending on the type of pemphigus. […] Pemphigus vulgaris blisters often start in the mouth but can develop on the skin later on. The skin may become so fragile that it peels off by rubbing a finger on it. Mucosal surfaces such as those of the nose, throat, eyes, and genitals may also be affected. Blisters form within the deep layer of the epidermis, and they are often painful.

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