Materiały źródłowe

• #1 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560860/

  Pemphigus vulgaris is a rare autoimmune disease that causes blistering on cutaneous and mucosal surfaces. As the most prevalent type of pemphigus, its onset typically occurs between ages 40 and 60, affecting both genders equally. […] Pemphigus vulgaris (PV) is an autoimmune disease that results in blisters on cutaneous and mucosal surfaces and is characterized primarily by acantholysis. […] PV is a blistering disease that initially presents on the oral mucosa in 80% of cases. These intraoral blisters often rupture, leaving painful erosions, which may affect patients’ oral intake, leading to potential malnutrition. Cutaneous lesions traditionally appear in patients with PV after the first oral blisters have presented. Vesicles, erosions, or bullae may appear on erythematous or normal-appearing skin. A Nikolsky sign is described as a blister formation with minor pressure or trauma and is seen in PV.

• #2 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.

• #3 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. […] 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.

• #4 Pemphigus Vulgaris – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.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.

• #5 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. […] 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. […] Mouth sores begin as blisters, which quickly burst causing the painful sores. […] 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.

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

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

  Pemphigus vulgaris. This is the most common form. It affects the moist parts of your body, like your mouth and genitals. Adults between ages 30 and 60 are most likely to get it. […] The first sign of a problem will typically be blisters in your mouth that peel easily. You may find it hard to swallow or eat. […] Next, blisters will often form on your skin or inside your genitals. They hurt, but they dont itch. […] 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.

• #7 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).

• #8 Pemphigus: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pemphigus

  Pemphigus vulgaris (PV) is the most common subset or variant and accounts for 70% of cases of pemphigus. […] Skin lesions: Skin blisters are flaccid rather than tense. Intact blisters may not be found. […] 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. […] The oral cavity is most often affected (almost all patients). Gingival, buccal and palatine lesions occur. […] Eating and drinking may become very uncomfortable. […] If successful, treatment should be tapered gradually, starting with the corticosteroids, followed by the adjuvant nonsteroidal agent. Generally, clinically significant improvement is expected within weeks for PF and within months for PV. […] 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.

• #9 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. […] The skin lesions start as thin-walled fragile blisters (collections of clear fluid within the skin) that burst easily, leaving raw areas known as erosions. Erosions are sore, burn-like areas that can ooze fluid or become crusty and infected and when they heal, the skin may be discoloured. […] Blisters in the mouth quickly burst to form erosions. There may be one or two, or several, that join together. […] 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.

• #10 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.

• #11 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).

• #12 Pemphigus vulgaris – British Skin Foundation

  https://knowyourskin.britishskinfoundation.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. […] The skin lesions start as thin-walled fragile blisters (collections of clear fluid within the skin) that burst easily, leaving raw areas known as erosions. […] Erosions are sore, burn-like areas that can ooze fluid or become crusty and infected and when they heal, the skin may be discoloured. […] Blisters in the mouth quickly burst to form erosions. There may be one or two, or several, that join together. […] 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.

• #13 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. […] 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. […] Mouth sores begin as blisters, which quickly burst causing the painful sores. […] 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.

• #14 Pemphigus – IPPF

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

  Pemphigus vulgaris usually involves a lot of blisters and erosions. […] The pain associated with mucosal involvement of pemphigus vulgaris can be severe. […] Oral pain is often augmented by chewing and swallowing, which may result in poor alimentation, weight loss, and malnutrition. […] Almost all pemphigus vulgaris patients will have some mucosal involvement. […] Because mucosal blisters erode quickly, erosions are often the only clinical findings. […] Many patients also have skin involvement. […] Blistering may be accompanied by severe pain, itching, burning, and stinging. […] If extensive, blistering can lead to life-threatening fluid loss, infection, and disfigurement. […] PV can also cause significant damage to the skin, including nail loss and pigmentary alteration, making timeliness of intervention and treatment essential to prevention of disability.

• #15 Pemphigus vulgaris (PV) | nidirect

  https://www.nidirect.gov.uk/conditions/pemphigus-vulgaris-pv

  Pemphigus vulgaris (PV) is a rare and serious condition causing painful blisters to develop on the skin and lining of the mouth, nose, throat and genitals. […] The blisters usually developing in the mouth first, before affecting the skin a few weeks or months later. […] There tends to be times when the blisters are severe (flare-ups), followed by periods when they heal and fade (remission). […] Blisters in the mouth often turn into painful sores, which can make eating, drinking and brushing teeth very difficult. […] The voice can become hoarse if they spread to the voice box (larynx). […] Sores on the skin can join together to form large areas of painful, raw-looking skin, before crusting over and forming scabs. They don’t usually leave any scars, although affected skin can occasionally become permanently discoloured.

• #16 Pemphigus vulgaris of the larynx, pharynx, and oral cavity | Iowa Head and Neck Protocols

  https://medicine.uiowa.edu/iowaprotocols/pemphigus-vulgaris-larynx-pharynx-and-oral-cavity

  Pemphigus vulgaris (PV) is an autoimmune, intraepithelial, bullous disease. It involves the skin and/or mucosa. Ig(G) antibodies to desmoglein 1 and 3 cause blisters due to acantholysis. It is a rare disease (0.1-0.5 cases/100,000 inhabitants/year) [Bascones-Martinez 2010]. […] PV usually begins with mucosal involvement with or without skin lesions. Oral lesions are hallmark of pemphigus vulgaris and occur in almost all cases [Mustafa 2015]. Oral lesions are the first manifestations in 50-90% of cases. They typically precede cutaneous lesions by 2-6 months [Bascones-Martinez 2010]. […] Oropharyngeal and laryngeal lesions may present with sore throat, dysphagia, hoarseness, and in severe cases airway impairment. […] Patients with oropharnygeal PV have high rates of achieving long-lasting clinical remission (94.6%) using conventional immunosuppresive therapy [Mignogna 2010].

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

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

  Mucous membranes of the oral cavity are involved in almost all patients with pemphigus vulgaris. Patients may have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal. Intact bullae are rare in the mouth. Erosions may be seen on any part of the oral cavity, and they may spread to involve the larynx, with subsequent hoarseness. In juvenile pemphigus vulgaris, stomatitis is the presenting complaint in more than 50% of cases. […] The primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base. Blisters are fragile and may rupture, producing painful erosions (the most common skin presentation). […] Pemphigus vulgaris involves mucosa in 50-70% of patients. This may limit oral intake secondary to dysphagia. Blistering and erosions secondary to the rupture of blisters may be painful and may limit the patient’s daily activities. Patients with pemphigus vulgaris typically heal without scarring unless the disease is complicated by severe secondary infection.

• #18 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  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. […] 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).

• #19 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).

• #20 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.

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

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

  Mucous membranes of the oral cavity are involved in almost all patients with pemphigus vulgaris. Patients may have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal. Intact bullae are rare in the mouth. Erosions may be seen on any part of the oral cavity, and they may spread to involve the larynx, with subsequent hoarseness. In juvenile pemphigus vulgaris, stomatitis is the presenting complaint in more than 50% of cases. […] The primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base. Blisters are fragile and may rupture, producing painful erosions (the most common skin presentation). […] Pemphigus vulgaris involves mucosa in 50-70% of patients. This may limit oral intake secondary to dysphagia. Blistering and erosions secondary to the rupture of blisters may be painful and may limit the patient’s daily activities. Patients with pemphigus vulgaris typically heal without scarring unless the disease is complicated by severe secondary infection.

• #22 Pemphigus vulgaris: Causes, treatment, and images

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

  The ruptured skin blisters may also limit a person’s daily activities. Skin lesions commonly affect the chest, scalp, back, and face. […] Other potential symptoms of pemphigus conditions include nail damage and nail loss, light sensitivity, eye problems, fatigue, and weakness. […] Another characteristic symptom in pemphigus conditions is the Nikolsky sign. This name derives from Dr. Pyotr Nikolsky and refers to when the top layers of the skin slip away from the lower layers in response to pressure. This sign is a useful diagnostic tool in identifying cases of blistering skin conditions. […] Pemphigus vulgaris is an autoimmune blistering skin condition. This means that the immune system mistakenly attacks healthy tissues in the skin. Specifically, the condition results from autoantibodies attacking proteins that help layers of skin stick together. This causes a buildup of fluid between the skin cells, which leads to the formation of blisters.

• #23 Pemphigus Vulgaris – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.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.

• #24 Pemphigus vulgaris: Causes, treatment, and images

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

  The ruptured skin blisters may also limit a person’s daily activities. Skin lesions commonly affect the chest, scalp, back, and face. […] Other potential symptoms of pemphigus conditions include nail damage and nail loss, light sensitivity, eye problems, fatigue, and weakness. […] Another characteristic symptom in pemphigus conditions is the Nikolsky sign. This name derives from Dr. Pyotr Nikolsky and refers to when the top layers of the skin slip away from the lower layers in response to pressure. This sign is a useful diagnostic tool in identifying cases of blistering skin conditions. […] Pemphigus vulgaris is an autoimmune blistering skin condition. This means that the immune system mistakenly attacks healthy tissues in the skin. Specifically, the condition results from autoantibodies attacking proteins that help layers of skin stick together. This causes a buildup of fluid between the skin cells, which leads to the formation of blisters.

• #25 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560860/

  Due to the painful nature of oral lesions, PV can impair nutritional status. Mucosal PV can be found in the conjunctiva, nasal mucosa, larynx, pharynx, esophagus, penis, vagina, and anus. […] Cutaneous lesions are commonly found on the face, trunk, groin, scalp, and armpits. PV usually spares the palms and soles. […] Blisters can heal without scarring but may result in changes in the pigment. Alopecia may be observed when PV affects the scalp. […] Another rare manifestation of PV is pemphigus herpetiformis, where PV can present with urticarial plaques and cutaneous vesicles arranged in a herpetiform or annular arrangement. […] Follow-up and response to treatment of PV should be monitored closely. PV is an active disease and often requires dose adjustments and changes in medications according to the response to treatment. Septicemia is the leading cause of death in PV.

• #26 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. […] The skin lesions start as thin-walled fragile blisters (collections of clear fluid within the skin) that burst easily, leaving raw areas known as erosions. Erosions are sore, burn-like areas that can ooze fluid or become crusty and infected and when they heal, the skin may be discoloured. […] Blisters in the mouth quickly burst to form erosions. There may be one or two, or several, that join together. […] 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.

• #27 Pemphigus vulgaris (PV) | nidirect

  https://www.nidirect.gov.uk/conditions/pemphigus-vulgaris-pv

  Pemphigus vulgaris (PV) is a rare and serious condition causing painful blisters to develop on the skin and lining of the mouth, nose, throat and genitals. […] The blisters usually developing in the mouth first, before affecting the skin a few weeks or months later. […] There tends to be times when the blisters are severe (flare-ups), followed by periods when they heal and fade (remission). […] Blisters in the mouth often turn into painful sores, which can make eating, drinking and brushing teeth very difficult. […] The voice can become hoarse if they spread to the voice box (larynx). […] Sores on the skin can join together to form large areas of painful, raw-looking skin, before crusting over and forming scabs. They don’t usually leave any scars, although affected skin can occasionally become permanently discoloured.

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

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

  Pemphigus vulgaris causes red and white fluid-filled blisters or open sores to form inside of your mouth. […] Pemphigus vulgaris causes blisters to form on your skin. A common location for blisters is near your groin and on the skin on your legs. […] 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. […] 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.

• #29 Pemphigus Vulgaris: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris

  Symptoms range in severity for each person diagnosed with pemphigus vulgaris and could include: Fluid-filled bump (blister) that has a red halo around the outside and a light pink to white center. The blister breaks open and leaks a clear fluid or lightly bleeds. Crust or scales form on or around the blister. The skin on or around the blister peels off. Open sores form when a blister breaks. Pain on or around the blisters. […] Symptoms of pemphigus vulgaris affect your skin and your mucous membranes. The most common places where you’ll experience symptoms include: Mouth and throat. Nose. Eyes. Genitals. Chest. Back. Arms and legs. Scalp. […] The length of time you need to heal varies for each person. On average, it could take several weeks for new blisters to stop forming after you start treatment. After that, it could take several months for your skin to heal.

• #30 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  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. […] 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).

• #31 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560860/

  Due to the painful nature of oral lesions, PV can impair nutritional status. Mucosal PV can be found in the conjunctiva, nasal mucosa, larynx, pharynx, esophagus, penis, vagina, and anus. […] Cutaneous lesions are commonly found on the face, trunk, groin, scalp, and armpits. PV usually spares the palms and soles. […] Blisters can heal without scarring but may result in changes in the pigment. Alopecia may be observed when PV affects the scalp. […] Another rare manifestation of PV is pemphigus herpetiformis, where PV can present with urticarial plaques and cutaneous vesicles arranged in a herpetiform or annular arrangement. […] Follow-up and response to treatment of PV should be monitored closely. PV is an active disease and often requires dose adjustments and changes in medications according to the response to treatment. Septicemia is the leading cause of death in PV.

• #32 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).

• #33 Pemphigus vulgaris – British Skin Foundation

  https://knowyourskin.britishskinfoundation.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).

• #34 Pemphigus Vulgaris: Signs, Symptoms, Causes, Diagnosis & Treatments

  https://www.apollo247.com/blog/article/pemphigus-vulgaris-signs-symptoms-causes-diagnosis-treatment

  In some cases, Pemphigus Vulgaris can extend to the eyes, leading to ocular involvement. This can cause symptoms such as eye irritation, redness, and discomfort. […] While less common, nasal involvement can occur in Pemphigus Vulgaris, leading to difficulty breathing. This may result from nasal mucosa blistering and swelling. […] Pemphigus Vulgaris is characterised by periods of disease activity followed by remission. During remission, patients experience a reduction in symptoms, and their skin and oral lesions heal. However, it’s important to note that Pemphigus Vulgaris can be a chronic condition with the potential for relapses, where new blistering may occur.

• #35 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).

• #36 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).

• #37 Pemphigus: Signs and symptoms

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

  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.

• #38 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  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. […] 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).

• #39 Pemphigus vulgaris – British Skin Foundation

  https://knowyourskin.britishskinfoundation.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).

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

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

  Severe symptoms of pemphigus include: Fever. Fatigue. Muscle weakness. Vision problems and light sensitivity. […] 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. […] If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms.

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

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

  Severe symptoms of pemphigus include: Fever. Fatigue. Muscle weakness. Vision problems and light sensitivity. […] 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. […] If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms.

• #42 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  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. […] 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).

• #43 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).

• #44 Pemphigus Vulgaris – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.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.

• #45 Pemphigus – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/454

  Pemphigus encompasses a group of potentially life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. […] Pemphigus vulgaris (PV) is the most common variant and affects skin and mucosa. […] Diagnosis is based on clinical findings of erosions (epidermal loss) and blisters of the skin, mucosa, or both. […] Pemphigus is associated with pain and impaired sleep. Swallowing difficulties are present if there is esophageal involvement. It is associated with increased rates of depression. […] Key diagnostic factors include chronic erosive blistering of the skin, mucosa, or both, chronic mouth erosions, painful lips, and shortness of breath. […] Other diagnostic factors include pruritic scalp, bloody nose, painful skin, dysphagia, pruritic skin, and conjunctivitis.

• #46 Pemphigus Vulgaris: Diagnosis & Procedure – UCF Health

  https://ucfhealth.com/our-services/dermatology/pemphigus-vulgaris/

  In some cases, scarring can occur, leaving marks of the disease on the body for life. Other long term effects may include stress, lack of sleep, lost time at work, and other intangible things. Even if the disease goes into remission, the individual may experience difficulty and stress when assuming normal life again. […] Upon noticing potential signs of pemphigus vulgaris, its crucial to seek help immediately. This autoimmune blistering disorder can quickly begin to impact daily life and grow life-threatening the longer it is left untreated.

• #47 Pemphigus vulgaris (PV) | nidirect

  https://www.nidirect.gov.uk/conditions/pemphigus-vulgaris-pv

  Pemphigus vulgaris (PV) is a rare and serious condition causing painful blisters to develop on the skin and lining of the mouth, nose, throat and genitals. […] The blisters usually developing in the mouth first, before affecting the skin a few weeks or months later. […] There tends to be times when the blisters are severe (flare-ups), followed by periods when they heal and fade (remission). […] Blisters in the mouth often turn into painful sores, which can make eating, drinking and brushing teeth very difficult. […] The voice can become hoarse if they spread to the voice box (larynx). […] Sores on the skin can join together to form large areas of painful, raw-looking skin, before crusting over and forming scabs. They don’t usually leave any scars, although affected skin can occasionally become permanently discoloured.

• #48 Pemphigus Vulgaris: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris

  Pemphigus vulgaris is a chronic condition, which means that your symptoms can return after you receive treatment. […] With treatment, you can control pemphigus vulgaris. The condition rarely affects your life expectancy. Symptoms can be temporarily disabling if they’re severe, which can prevent you from going about your routine or going to work. Some people have trouble sleeping and may experience weight loss if the sores in their mouth prevent them from eating normally. […] Without treatment, symptoms can spread throughout your body and cause life-threatening complications like malnutrition, dehydration and sepsis. A severe, untreated infection is the most common complication of untreated pemphigus vulgaris. […] Pemphigus vulgaris is a chronic condition and you could experience symptoms throughout your life. Most often, symptoms will begin between the ages of 40 to 60. Symptoms can unexpectedly arise (flare) and go away. Treatment can help reduce your symptoms.

• #49 Pemphigus Vulgaris: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris

  Pemphigus vulgaris is a chronic condition, which means that your symptoms can return after you receive treatment. […] With treatment, you can control pemphigus vulgaris. The condition rarely affects your life expectancy. Symptoms can be temporarily disabling if they’re severe, which can prevent you from going about your routine or going to work. Some people have trouble sleeping and may experience weight loss if the sores in their mouth prevent them from eating normally. […] Without treatment, symptoms can spread throughout your body and cause life-threatening complications like malnutrition, dehydration and sepsis. A severe, untreated infection is the most common complication of untreated pemphigus vulgaris. […] Pemphigus vulgaris is a chronic condition and you could experience symptoms throughout your life. Most often, symptoms will begin between the ages of 40 to 60. Symptoms can unexpectedly arise (flare) and go away. Treatment can help reduce your symptoms.

• #50 Pemphigus Vulgaris: Cause, Types, and Symptoms

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

  Pemphigus vulgaris is a lifelong condition. It cannot be cured. However, most people go into remission after receiving corticosteroids. Improvement is usually noticed within days of starting the corticosteroids. […] The blisters will heal slowly, especially the ones in the mouth. On average, the blisters stop forming in two to three weeks. Healing of the blisters takes six to eight weeks on average. However, full healing can sometimes take years. Some individuals may need to stay on a low dose of the medication for life.

• #51 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.

• #52 Pemphigus Vulgaris – UF Health

  https://ufhealth.org/conditions-and-treatments/pemphigus-vulgaris

  About 50% of people with this condition first develop painful blisters and sores in the mouth. This is followed by skin blisters. Skin sores may come and go. […] The skin sores may be described as: Draining, Oozing, Crusting, Peeling or easily detached. […] They may be located: In the mouth and down the throat, On the scalp, trunk, or other skin areas. […] Without treatment, this condition can be life threatening. Severe infection is the most frequent cause of death. […] With treatment, the disorder tends to be chronic. Side effects of treatment may be severe or disabling.

• #53

  https://111.wales.nhs.uk/encyclopaedia/p/article/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.

• #54 Pemphigus vulgaris: Causes, treatment, and images

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

  After initial treatment, it may take 2-3 weeks for blister development to stop and 6-8 weeks for healing to occur. Treatment will then typically involve slowly decreasing medications to allow a person to manage the condition. […] Aside from the severe pain, extensive blistering may also lead to infections, life-threatening fluid loss, significant skin damage, nail changes, pigment changes, and sepsis. […] Pemphigus vulgaris is a rare autoimmune skin blistering condition. The characteristic feature of this condition is blistering of the mucous membranes, most commonly within the mouth. It occurs when the immune system mistakenly attacks proteins in the skin, causing blisters to form.

• #55 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).

• #56 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.

• #57 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560860/

  A retrospective cohort study by Kridin et al. in Israel found that survival rates were lower in patients with PV compared with the general population. Patients who were diagnosed with PV at an older age had a lower survival rate. The median overall survival from the point of diagnosis was 10.1 years (0.2 to 29.8 years). There was not a statistically significant survival difference between men and women.

• #58 About Pemphigus vulgaris (PV)

  https://medically.roche.com/global/en/microsites/about-pv.html

  PV usually manifests as painful lesions of the oral mucosa, which can be buccal and/or gingival, and can interfere with eating if they are persistent. […] The blisters that develop in PV are flaccid in nature making them fragile and prone to rupturing. The blisters rupture quickly, particularly in the mouth, resulting in painful lesions or erosions that may spread due to continued shedding of the epithelium. […] In the majority (50-70%) of cases, the first sign of PV is the development of blisters in the mouth, commonly on the inside of the cheeks. Inflammation of the mouth and lips (stomatitis) may also indicate the onset of PV. Patients may have difficulty swallowing due to persistent and painful lesions which are slow to heal. Over time this may lead to weight loss and malnutrition. […] PV is a chronic disease that if left untreated can quickly progress to severe disease with multiple erosions that are susceptible to infections and dehydration. […] PV patients are reported to have a 3-times higher risk of mortality than the general population.

• #59 Pemphigus Vulgaris

  https://ketteringhealth.org/conditions/pemphigus-vulgaris/

  Pemphigus vulgaris often starts in the mouth. Symptoms include: Blisters on otherwise healthy skin […] Blisters that are easy to burst […] Affected skin that peels easily when rubbed […] Pain at blister. Blisters may be painful. They may heal and leave dark patches on the skin for months. Most people with pemphigus vulgaris feel better with treatment. Without treatment, the condition can lead to severe pain and infection. Call your healthcare provider if you notice painful, soft blistering on your skin or mucous membranes. Treatment will prevent the blisters from spreading, becoming infected, and getting worse. Some people find that stress and eating certain spicy, acidic, or citrus foods make pemphigus vulgaris worse. This may be true even during treatment.

• #60 Pemphigus Vulgaris – UF Health

  https://ufhealth.org/conditions-and-treatments/pemphigus-vulgaris

  About 50% of people with this condition first develop painful blisters and sores in the mouth. This is followed by skin blisters. Skin sores may come and go. […] The skin sores may be described as: Draining, Oozing, Crusting, Peeling or easily detached. […] They may be located: In the mouth and down the throat, On the scalp, trunk, or other skin areas. […] Without treatment, this condition can be life threatening. Severe infection is the most frequent cause of death. […] With treatment, the disorder tends to be chronic. Side effects of treatment may be severe or disabling.

• #61 Pemphigus: Symptoms, Causes, Diagnosis, Treatment, and Coping

  https://www.verywellhealth.com/pemphigus-4691395

  Pemphigus vulgaris will generally affect mucosal membranes of the mouth first, causing multiple ulcers that can persist for weeks and months. In some cases, the oral lesions may be the only symptom. In others, blisters may develop on the skin, most predominately the upper chest, back, scalp, and face. […] The blisters are generally ill-defined and easily ruptured. They will often consolidate into larger blisters and cause extensive peeling and oozing. The blisters are almost invariably painful, and, depending on their location, can be itchy or non-itchy. […] If left untreated, pemphigus can gradually spread and involve greater amounts of tissue. This can lead to potentially life-threatening complications, including: Malnutrition (due to painful mouth or throat sores), Fluid loss and severe dehydration, Infection, Sepsis and septic shock.

• #62 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  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. […] 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).

• #63 Pemphigus: Symptoms, Causes, Diagnosis, Treatment, and Coping

  https://www.verywellhealth.com/pemphigus-4691395

  Pemphigus vulgaris will generally affect mucosal membranes of the mouth first, causing multiple ulcers that can persist for weeks and months. In some cases, the oral lesions may be the only symptom. In others, blisters may develop on the skin, most predominately the upper chest, back, scalp, and face. […] The blisters are generally ill-defined and easily ruptured. They will often consolidate into larger blisters and cause extensive peeling and oozing. The blisters are almost invariably painful, and, depending on their location, can be itchy or non-itchy. […] If left untreated, pemphigus can gradually spread and involve greater amounts of tissue. This can lead to potentially life-threatening complications, including: Malnutrition (due to painful mouth or throat sores), Fluid loss and severe dehydration, Infection, Sepsis and septic shock.

• #64 Pemphigus – IPPF

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

  Pemphigus vulgaris usually involves a lot of blisters and erosions. […] The pain associated with mucosal involvement of pemphigus vulgaris can be severe. […] Oral pain is often augmented by chewing and swallowing, which may result in poor alimentation, weight loss, and malnutrition. […] Almost all pemphigus vulgaris patients will have some mucosal involvement. […] Because mucosal blisters erode quickly, erosions are often the only clinical findings. […] Many patients also have skin involvement. […] Blistering may be accompanied by severe pain, itching, burning, and stinging. […] If extensive, blistering can lead to life-threatening fluid loss, infection, and disfigurement. […] PV can also cause significant damage to the skin, including nail loss and pigmentary alteration, making timeliness of intervention and treatment essential to prevention of disability.

• #65 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  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. […] 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).

• #66 Pemphigus Vulgaris: Diagnosis & Procedure – UCF Health

  https://ucfhealth.com/our-services/dermatology/pemphigus-vulgaris/

  In some cases, scarring can occur, leaving marks of the disease on the body for life. Other long term effects may include stress, lack of sleep, lost time at work, and other intangible things. Even if the disease goes into remission, the individual may experience difficulty and stress when assuming normal life again. […] Upon noticing potential signs of pemphigus vulgaris, its crucial to seek help immediately. This autoimmune blistering disorder can quickly begin to impact daily life and grow life-threatening the longer it is left untreated.

• #67 Pemphigus vulgaris – British Skin Foundation

  https://knowyourskin.britishskinfoundation.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).

• #68 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.

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

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

  A rare variant called pemphigus vegetans occurs primarily in intertriginous areas and oral mucosa with vegetating cauliflower-like plaques. […] The primary lesions of pemphigus vulgaris are flaccid bullae. […] In pemphigus vulgaris, painful erosions on the skin and mucosa are common. […] Pemphigus lesions typically appear first in the mouth, where they rupture and remain as chronic, painful erosions for variable periods. Oral lesions often precede cutaneous lesions. […] 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.

• #70 Pemphigus Vulgaris – Skin Disorders – Merck Manual Consumer Version

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

  Without treatment, pemphigus vulgaris is often fatal, usually within 5 years. Treatment greatly improves survival, but the chance of dying is still about twice that of the general population. The risk of death and severe complications tends to be higher if people have widespread pemphigus vulgaris, require high doses of corticosteroids or other drugs that suppress the immune system to control the disorder, or have other serious disorders.

• #71 About Pemphigus vulgaris (PV)

  https://medically.roche.com/global/en/microsites/about-pv.html

  PV usually manifests as painful lesions of the oral mucosa, which can be buccal and/or gingival, and can interfere with eating if they are persistent. […] The blisters that develop in PV are flaccid in nature making them fragile and prone to rupturing. The blisters rupture quickly, particularly in the mouth, resulting in painful lesions or erosions that may spread due to continued shedding of the epithelium. […] In the majority (50-70%) of cases, the first sign of PV is the development of blisters in the mouth, commonly on the inside of the cheeks. Inflammation of the mouth and lips (stomatitis) may also indicate the onset of PV. Patients may have difficulty swallowing due to persistent and painful lesions which are slow to heal. Over time this may lead to weight loss and malnutrition. […] PV is a chronic disease that if left untreated can quickly progress to severe disease with multiple erosions that are susceptible to infections and dehydration. […] PV patients are reported to have a 3-times higher risk of mortality than the general population.

• #72 Pemphigus: Symptoms and Treatment | Doctor

  https://patient.info/doctor/pemphigus

  Pemphigus vulgaris (PV) is the most common subset or variant and accounts for 70% of cases of pemphigus. […] Skin lesions: Skin blisters are flaccid rather than tense. Intact blisters may not be found. […] 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. […] The oral cavity is most often affected (almost all patients). Gingival, buccal and palatine lesions occur. […] Eating and drinking may become very uncomfortable. […] If successful, treatment should be tapered gradually, starting with the corticosteroids, followed by the adjuvant nonsteroidal agent. Generally, clinically significant improvement is expected within weeks for PF and within months for PV. […] 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.

• #73 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).

• #74 Pemphigus Vulgaris – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560860/

  A retrospective cohort study by Kridin et al. in Israel found that survival rates were lower in patients with PV compared with the general population. Patients who were diagnosed with PV at an older age had a lower survival rate. The median overall survival from the point of diagnosis was 10.1 years (0.2 to 29.8 years). There was not a statistically significant survival difference between men and women.

• #75 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).

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

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

  Pemphigus vulgaris. This is the most common form. It affects the moist parts of your body, like your mouth and genitals. Adults between ages 30 and 60 are most likely to get it. […] The first sign of a problem will typically be blisters in your mouth that peel easily. You may find it hard to swallow or eat. […] Next, blisters will often form on your skin or inside your genitals. They hurt, but they dont itch. […] 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.

• #77 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.

• #78 Pemphigus Vulgaris: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris

  Pemphigus vulgaris is a chronic condition, which means that your symptoms can return after you receive treatment. […] With treatment, you can control pemphigus vulgaris. The condition rarely affects your life expectancy. Symptoms can be temporarily disabling if they’re severe, which can prevent you from going about your routine or going to work. Some people have trouble sleeping and may experience weight loss if the sores in their mouth prevent them from eating normally. […] Without treatment, symptoms can spread throughout your body and cause life-threatening complications like malnutrition, dehydration and sepsis. A severe, untreated infection is the most common complication of untreated pemphigus vulgaris. […] Pemphigus vulgaris is a chronic condition and you could experience symptoms throughout your life. Most often, symptoms will begin between the ages of 40 to 60. Symptoms can unexpectedly arise (flare) and go away. Treatment can help reduce your symptoms.

• #79 Pemphigus Vulgaris: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris

  Pemphigus vulgaris is a chronic condition, which means that your symptoms can return after you receive treatment. […] With treatment, you can control pemphigus vulgaris. The condition rarely affects your life expectancy. Symptoms can be temporarily disabling if they’re severe, which can prevent you from going about your routine or going to work. Some people have trouble sleeping and may experience weight loss if the sores in their mouth prevent them from eating normally. […] Without treatment, symptoms can spread throughout your body and cause life-threatening complications like malnutrition, dehydration and sepsis. A severe, untreated infection is the most common complication of untreated pemphigus vulgaris. […] Pemphigus vulgaris is a chronic condition and you could experience symptoms throughout your life. Most often, symptoms will begin between the ages of 40 to 60. Symptoms can unexpectedly arise (flare) and go away. Treatment can help reduce your symptoms.

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