Materiały źródłowe

• #1 Bullous Pemphigoid – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK535374/

  Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disorder, representing 80% of subepidermal immunobullous cases. It most commonly affects elderly patients between the ages of 60 and 80. […] While the clinical presentation of bullous pemphigoid is broad, the immunobullous skin disorder characteristically presents with tense bullae and intense generalized pruritus. In atypical cases, bullous lesions may be absent, and these cases require a high degree of clinical suspicion. […] The bullous phase characteristically presents with vesicles and bullae on a normal or erythematous skin background. The blisters are tense, up to 1 to 4 centimeters in diameter, and sometimes hemorrhagic. They typically contain clear fluid and may persist for several days before leaving erosions and crusts.

• #2 Bullous pemphigoid – Wikipedia

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

  Bullous pemphigoid is an autoimmune pruritic skin disease that typically occurs in people aged over 60, that may involve the formation of blisters (bullae) in the space between the epidermal and dermal skin layers. […] Clinically, the earliest lesions may appear as a hives-like red raised rash, but could also appear dermatitic, targetoid, lichenoid, nodular, or even without a rash (essential pruritus). Bullous Pemphigoid, is characterized by the subepidermal blisters resulting in tense and less fragile bullae. […] Tense bullae eventually erupt, most commonly at the inner thighs and upper arms, but the trunk and extremities are frequently both involved. Any part of the skin surface can be involved. Oral lesions are present in a minority of cases. […] The disease may be acute, but can last from months to years with periods of exacerbation and remission.

• #3 Bullous pemphigoid – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/symptoms-causes/syc-20350414

  People with bullous pemphigoid may develop many blisters. When the blisters break, they leave a sore that usually heals without a scar. […] Bullous pemphigoid is a rare skin condition that causes large fluid-filled blisters. They often appear on the skin near creases, such as the upper thighs and armpits. Sometimes, people get a rash instead of blisters. The affected areas may be painful and are usually very itchy. Blisters or sores also might form in the mouth, but this is rare. […] Bullous pemphigoid often goes away on its own in a few months, but it may take as many as five years to go away for good. Treatment usually helps heal the blisters and prevent new ones from forming. […] The symptoms of bullous pemphigoid may include: Itching, which can start weeks or months before blisters form. Large blisters that don’t break easily, often found along skin folds. On brown and Black skin the blisters may be dark pink, brown or black. On white skin they may be yellow, pink or red. Pain. A rash. Small blisters or sores in the mouth or other mucous membranes. This is a symptom of a rare type of the disease called mucous membrane pemphigoid. […] Bullous pemphigoid is most common in people over age 60, and the risk increases with age. The condition can be life-threatening for older people who have other conditions at the same time.

• #4 Bullous Pemphigoid – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK535374/

  Bullous pemphigoid typically resolves spontaneously in a few months but can remain for up to 5 years. […] Bullous pemphigoid is typically a chronic disease with unpredictable exacerbations, with up to 30% of affected patients relapsing within the first year. When treatment is discontinued, up to 50% of patients may relapse within the first 3 months of discontinuation. The mortality rate is increased in bullous pemphigoid due to the elderly population being affected, with a death rate of approximately 10% to 40%.

• #5 Bullous pemphigoid – Wikipedia

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

  Bullous pemphigoid goes through two distinct phases: The non-bulbous and the bullous phase. […] Non-bulbous phase, involves mild to severe intractable pruritic skin due to the non-specificity of the disease, involving eczematous or urticarial lesions. This phase of Bullous pemphigoid can occur over the time period of weeks to months. […] In the bullous phase, tense blisters form in an annular shape and are filled with clear fluid. This occurs on the abdomen, limbs and lower trunk. These blisters average between 1cm-4cm in diameter. […] Bulbous pemphigoid may be self-resolving in a period ranging from several months to many years even without treatment.

• #6 Bullous pemphigoid: Signs and symptoms

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

  Bullous pemphigoid is a disease that causes blisters, which can develop anywhere on the skin. […] Most people develop blisters on one or more of the following areas: Arms, Armpits, Legs, Abdomen, Groin, Mouth. […] The blisters may appear on a few or many areas of the skin. When the blisters develop on many areas, the medical term for this is widespread. Widespread blisters appear in many areas like the arms, back, chest, and legs. […] Often beginning after 60 years of age, this disease usually causes a cycle of blisters. As the old blisters clear, new blisters often form. […] While bullous pemphigoid is a blistering disease, research shows that about 20% of people who develop this disease never get blisters. Instead, they can have itching, a rash, or both. […] Before the blisters appear for the first time, your skin may itch. Some people have mildly itchy skin. For others, the itch can be intense. The itch can begin weeks (or months) before blisters appear.

• #7 Bullous Pemphigoid – Dermatologic Disorders – Merck Manual Professional Edition

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

  Pruritus is the first symptom of bullous pemphigoid. Skin lesions may not develop for several years. Often, characteristic tense bullae develop on skin of the trunk and in the flexural and intertriginous areas. Bullae may develop on normal-appearing skin or may be preceded by erythematous or urticarial-appearing plaques. Localized disease may occur at trauma sites, stomas, and anogenital and lower leg areas. Dyshidrotic pemphigoid is a rare form of bullous pemphigoid that affects the hands and feet and can look like dyshidrotic dermatitis (a form of hand and foot dermatitis) on the palms. Bullae usually do not rupture, but those that do often rapidly heal. […] Symptoms usually lessen within months, but treatment is sometimes needed for several years.

• #8 Bullous Pemphigoid: Know the Facts

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

  Bullous pemphigoid is a rare autoimmune disease that affects your skin. Its a chronic disease that causes itchy, fluid-filled blisters to form all over your body. […] The primary symptoms for bullous pemphigoid are large, fluid-filled blisters on the skin, often in creases or skin folds. The blisters are almost a half-an-inch in size or larger and are most often on your: Arms, Groin, Legs, Armpits, Mouth, Abdomen (Belly area). […] Other symptoms can include: Itchy skin for weeks or even months before the blisters appear. The itch can be mild or intense. It also might only be in some areas, not all over. Red or darker skin around the blisters. Itchy welts or a rash that might look like hives. This can last for days or weeks. Eczema-like rash. Solid blisters that can be as large as 2 inches across. They feel tight and dont rupture or break easily.

• #9 Bullous pemphigoid – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/453?locale=it

  Bullous pemphigoid typically occurs in older people and has a distinctive clinical appearance. There are several clinical variants, one of which occurs in childhood. […] In the prodromal, nonbullous phase, pruritus of variable intensity may be accompanied by eczematous or urticarial lesions for weeks or months. […] In the bullous stage, characteristic, tense vesicles or bullae develop on apparently normal or erythematous skin of the pre-existing eczematous or urticarial eruption. If the blisters burst, the eroded, crusty areas slowly heal to leave postinflammatory hyperpigmentation. […] The lesions are usually symmetric and favor the flexural aspects of the extremities, lower trunk, and abdomen.

• #10 Bullous pemphigoid

  https://www.nhs.uk/conditions/bullous-pemphigoid/

  Bullous pemphigoid usually starts with an itchy, raised rash on the skin, usually the backs of the knees, insides of the armpits and elbows, hands, feet or tummy. […] After a few weeks, small blisters may appear. […] A large fluid-filled blister next to a smaller blood-filled blister on the wrist of a person with white skin. The blisters can grow quite big and may contain blood. […] Some people may also get blisters in their mouth, which can cause problems swallowing (dysphagia). […] Not everyone gets blisters. If you do, they may keep coming and going for months or years. […] Bullous pemphigoid eventually goes away on its own.

• #11 Bullous pemphigoid

  https://www.pcds.org.uk/clinical-guidance/bullous-pemphigoid1

  Bullous pemphigoid is an uncommon blistering condition of the elderly, which often starts with itch and urticated and erythematous lesions. Later, large tense blisters develop on both erythematous and on normal skin and there may be mucosal involvement with blisters and erosions. […] Itch is a common feature, and may precede the rash by several weeks or months. […] Bullous pemphigoid commonly starts with itching and fixed urticarial-like lesions or occasionally an eczematous rash, which predominantly affects the limbs. […] The itch may precede the rash for several months. […] An urticarial prodrome usually lasts 1-3 weeks before the blisters arise, whereas an eczematous prodrome may precede the blisters by several months. […] Generalised bullous pemphigoid is the most common form, blisters can form anywhere and are especially widespread on the trunk, proximal limbs and flexures. Once blisters arise they can become widespread within a matter of days.

• #12 Bullous Pemphigoid: Know the Facts

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

  About 20% of people with bullous pemphigoid dont get blisters, but they have some of the other symptoms. […] There is also a subtype of bullous pemphigoid that causes blisters to appear on the palms of your hands and soles of your feet. […] It can take several years for bullous pemphigoid to go away completely. In the meantime, your doctor will monitor your progress to see how the treatment is working, and may switch drugs if needed. Some medications can have serious side effects, so report anything unusual to your doctor. You might need regular tests, like a bone density (DEXA) scan, blood pressure monitoring, or blood tests to make sure that you arent having any hidden side effects. […] Bullous pemphigoid does go away, but it can take as long as five years in some people.

• #13 Strategies to improve outcomes of bullous pemphigoid | CCID

  https://www.dovepress.com/strategies-to-improve-outcomes-of-bullous-pemphigoid-a-comprehensive-r-peer-reviewed-fulltext-article-CCID

  Bullous pemphigoid (BP) is the most frequent autoimmune bullous disease mainly affecting elderlies. Diagnosis usually results from clinical features, histological examination, and the quantification of circulating typical autoantibodies. […] BP treatment and management still represents a challenge due to the higher frequency of several comorbidities in this group of patients, which may also be linked to a reduced tolerance to BP treatments. […] Hence, an early diagnosis and a prompt correct treatment are mandatory to reach better clinical outcomes and improve as much as possible BP outcomes. […] The BP clinical presentation can be highly variable, especially in the early stages of the disease or in atypical variations that lack the usual blistering lesions. […] First signs and symptoms in the non-bullous phase, are commonly nonspecific, usually presenting with pruritus alone or accompanied by several types of lesions, including papular, urticarial, and eczematous lesions: these non-specific manifestations results in a difficult diagnosis.

• #14 Bullous Pemphigoid – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK535374/

  Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disorder, representing 80% of subepidermal immunobullous cases. It most commonly affects elderly patients between the ages of 60 and 80. […] While the clinical presentation of bullous pemphigoid is broad, the immunobullous skin disorder characteristically presents with tense bullae and intense generalized pruritus. In atypical cases, bullous lesions may be absent, and these cases require a high degree of clinical suspicion. […] The bullous phase characteristically presents with vesicles and bullae on a normal or erythematous skin background. The blisters are tense, up to 1 to 4 centimeters in diameter, and sometimes hemorrhagic. They typically contain clear fluid and may persist for several days before leaving erosions and crusts.

• #15 Strategies to improve outcomes of bullous pemphigoid | CCID

  https://www.dovepress.com/strategies-to-improve-outcomes-of-bullous-pemphigoid-a-comprehensive-r-peer-reviewed-fulltext-article-CCID

  The development of more typical vesicles and bullae is the main characteristic of the bullous stage of BP: in classic BP tense bullae, varying from 1 to 3 cm of diameter, and usually appearing on erythematous or normal skin. […] BP typically presents with a chronic-remitting course, being self-limited in a few years. […] Although skin or mucosal lesions are rarely fatal, patients have a six-fold greater death rate than a healthy, age-matched population. […] Regarding BP prognosis, clinical risk factors linked to a higher mortality include older age, and neurological disorders with a higher incidence in elderly patients, such as dementia (including Alzheimers disease). […] An early diagnosis, and consequently correct management, monitoring, and assessment of patients suffering from BP are crucial to improving as much as possible the prognosis of these patients.

• #16 Pemphigoid: Types, Causes, and Symptoms

  https://www.healthline.com/health/bullous-pemphigoid

  In cases of bullous pemphigoid the most common of the three types the skin blistering happens most commonly on the arms and legs where movement occurs. This includes the areas around the joints and on the lower abdomen. […] The most common symptom of pemphigoid is blistering that occurs on the arms, legs, abdomen, and mucous membranes. Hives and itching are also common. The blisters have certain characteristics, regardless of where on the body they form: a red rash develops before the blisters, the blisters are large and filled with fluid thats usually clear, but may contain some blood, the blisters are thick and dont rupture easily, the skin around the blisters may appear normal, or slightly red or dark, ruptured blisters are usually sensitive and painful. […] With comprehensive treatment, the outlook for pemphigoid is good. Most people respond well to medication. The disease will often go away after a few years of treatment. But pemphigoid may return at any time, even with proper treatment.

• #17 Bullous pemphigoid

  https://www.pcds.org.uk/clinical-guidance/bullous-pemphigoid1

  Crops of large, tense, fluid-filled blisters, which may arise from normal-looking or erythematous skin. […] They are generally clear but can be cloudy or blood-stained. […] Blisters may remain intact for several days. […] Lesions heal quickly and without scarring. […] Mucosal involvement with small blisters is uncommon, and tends not to be clinically significant when it does occur. The blisters mainly affect the palate of the mouth. […] Bullous pemphigoid is a serious condition, with an average duration of 3-6 years. It is occasionally fatal. Factors associated with a worse prognosis include generalised disease, low albumin, and high dose steroids. In contrast, localised bullous pemphigoid has a very good prognosis.

• #18 Bullous pemphigoid | Altru Health System

  https://www.altru.org/health-library/conditions/bullous-pemphigoid

  Bullous pemphigoid is a rare skin condition that causes large fluid-filled blisters. They often appear on the skin near creases, such as the upper thighs and armpits. Sometimes, people get a rash instead of blisters. The affected areas may be painful and are usually very itchy. Blisters or sores also might form in the mouth, but this is rare. […] The symptoms of bullous pemphigoid may include: Itching, which can start weeks or months before blisters form. Large blisters that don’t break easily, often found along skin folds. On brown and Black skin the blisters may be dark pink, brown or black. On white skin they may be yellow, pink or red. Pain. A rash. Small blisters or sores in the mouth or other mucous membranes. This is a symptom of a rare type of the disease called mucous membrane pemphigoid.

• #19 Bullous Pemphigoid: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/15855-bullous-pemphigoid

  Bullous pemphigoid causes itchy, hive-like welts on your skin. […] Bullous pemphigoid appears as itchy welts that look like hives or multiple, itchy blisters (bullae). It most commonly appears on your arms, legs, abdomen, groin, and mouth. […] The blisters typically appear along the creases of your skin. They usually aren’t painful, but they may break open and become a painful sore or ulcer. […] Bullous pemphigoid usually clears up on its own after about five years. It generally responds well to appropriate treatment. You may have flare-up and times where it seems to go away (remission). During remission periods, you may stop your treatment. […] If your blisters pop and become infected, you’re at risk of developing sepsis, which is a life-threatening condition.

• #20 Bullous Pemphigoid: Causes, Symptoms, and Treatment — DermNet

  https://dermnetnz.org/topics/bullous-pemphigoid

  Bullous pemphigoid causes severe itch and (usually) large, tense bullae (fluid-filled blisters), which rupture forming crusted erosions. […] Bullous pemphigoid typically involves the flexor aspects of the limbs. It may be localised to one area, or widespread on the trunk and proximal limbs. […] Some patients have a diagnosis of bullous pemphigoid made despite not having any bullae (non-bullous pemphigoid). This can affect any site on the body. […] Bullous pemphigoid can be a serious disease, particularly when widespread or resistant to treatment. Morbidity and mortality result from: bacterial staphylococcal and streptococcal skin infection, and sepsis; viral infection with herpes simplex, varicella or herpes zoster; complications of treatment; underlying and associated diseases. […] Treatment for bullous pemphigoid is usually needed for several years. In many cases, the pemphigoid eventually completely clears up, and the treatment can be stopped. If blisters recur, treatment can be started again.

• #21 Bullous Pemphigoid – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/blistering-diseases/bullous-pemphigoid

  Bullous pemphigoid is an autoimmune disease that causes blistering of the skin. […] Symptoms of Bullous Pemphigoid […] Itching is often the first symptom of bullous pemphigoid. Blisters may not appear for several years. Before blisters appear, large, raised areas may develop, sometimes looking like hives. The immune system eventually forms antibodies directed against the skin, resulting in large, tense, and very itchy blisters surrounded by areas of normal-appearing skin or red, inflamed skin. The blisters usually do not burst, but those that do often rapidly heal. […] Blisters commonly develop on the parts of the body that are able to flex, such as the backs of the knees, the underarms, the insides of the elbows, and the groin. They sometimes develop on areas of skin that have been injured, the anus or genitals, the lower legs, and on areas where an artificial opening to the outside of the body (such as a stoma in the abdomen) exists. A rare form of bullous pemphigoid affects the hands and feet and can look like a form of hand and foot dermatitis. Blisters in the mouth are rare. […] Bullous pemphigoid is a chronic disease. Although drugs are helpful, they may cause side effects. With treatment, symptoms typically go away within months, but treatment is sometimes needed for several years.

• #22

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6486083/

  These lesions affect mainly the axillary folds, lower abdomen, inguinal areas and inner parts of the thighs. […] Mucosal involvement is not often detected, and is reported in 10-30% of the cases with oral, esophageal and genital lesions. […] Nonbullous BP at the onset of the disease develop blisters during their follow-up, and rarely display mucosal lesions. […] The most frequent clinical findings include pruritus (100%) with erythematous urticarial lesions (52.3%), excoriations (22.7%), or papules and nodules (20.5%). […] BP is a chronic relapsing disease that affects elderly individuals often with multiple comorbidities and functional impairment. […] A correlation between disease activity and serum levels of anti-BP180 IgG3 and IgE was demonstrated and these may be useful biomarkers to guide steroid tapering and to determine the set of patients that can benefit from the treatment with omalizumab.

• #23 Bullous pemphigoid: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000883.htm

  Bullous pemphigoid is a skin disorder characterized by blisters. […] Symptoms come and go. The condition often goes away within 5 years. […] Most people with this disorder have itchy skin that may be severe. In most cases, there are blisters, called bullae. […] Blisters are usually located on the arms, legs, or middle of the body. In rare cases, blisters can form in the mouth. […] The blisters may break open and form open sores (ulcers). […] Bullous pemphigoid usually responds well to treatment. The medicine can often be stopped after several years. The disease sometimes returns after treatment is stopped.

• #24 Bullous Pemphigoid: Symptoms and Treatment | Doctor

  https://patient.info/doctor/bullous-pemphigoid-pro

  Rash. An urticarial or erythematous rash may precede the appearance of the blisters. […] Blisters or bullae, which are typical and often occur in skin flexures. Depending on the form of bullous pemphigoid these may affect a single site or be more widespread. […] Mucous membranes. Blisters appear mainly on palatal mucosa. They may be clinically insignificant but can cause problems with dysphagia. […] Bullous pemphigoid is a chronic inflammatory disease which can persist with remissions and exacerbations for months or years. […] There is twice as high a risk of death compared with age-matched controls. […] Elderly patients are also particularly at risk from the immunosuppressive effects of therapy, which may place them at greater risk of life-threatening events than the disease itself.

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

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

  If you have unexplained blisters appearing on your body, pemphigus and pemphigoid could be to blame. These conditions are caused by an immune system gone awry and manifest as blisters in the skin or membranes such as the mouth, nose and eyes, says Yale Medicine dermatologist Mary M. Tomayko, MD, PhD. 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.

• #26 Bullous pemphigoid: Signs and symptoms

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

  The blisters can appear on skin with (or without) a rash. […] In time, the blisters collapse and crust over. When a blister clears, the skin tends to feel raw and tender. […] As the blisters go away, you may see spots of discolored skin (or tiny, raised, white bumps) where you had the blisters. […] Bullous pemphigoid is a chronic disease, which means it can last a long time. For some people, new blisters will continue to appear for years or a lifetime. It’s also possible that the blisters will go away on their own in a few months. Treatment can help reduce flare-ups and ease the itch.

• #27 Bullous Pemphigoid: What It Is and How to Treat It | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/patient-education/bullous-pemphigoid-what-it-and-how-treat-it

  Bullous pemphigoid is a rare skin condition. It causes large fluid-filled blisters to form on your skin. They often start on areas of your skin that flex (bend). These areas include your arms, armpits, legs, lower abdomen (belly), and groin (the area between your belly and thigh. […] The most common symptom of bullous pemphigoid is small patches of itchy skin. This is usually the first symptom. […] Some people may get blisters after a week or more, while others may get blisters months later. These blisters are often itchy, but not painful. They’re mostly firm and dome shaped. […] The skin over your blisters is very strong. Because of this, it can take a few days for your blister to burst (open). After your blister bursts, a raw patch of skin is left. This patch of skin will heal within 7 to 10 days.

• #28 Bullous Pemphigoid: Causes, Symptoms, and Treatment

  https://patient.info/skin-conditions/bullous-pemphigoid-leaflet

  Bullous pemphigoid is a skin disease that can cause a rash, itching and blisters. It mainly affects people aged over 70. Treatment usually works well to control symptoms. Treatment is usually with steroid creams or medicines, but sometimes other medicines may be used. The condition tends to go away after 3-6 years and then treatment can be stopped. Sometimes the disease persists. […] Symptoms of bullous pemphigoid include: Areas of itchy skin. Pink rash which can look like eczema or urticaria. Firm and dome-shaped fluid-filled blisters. Small blisters inside the mouth or on the lips. The itch or rash can develop weeks or months before the blisters appear. […] Bullous pemphigoid usually goes away after 3-6 years. Treatment can then be stopped. In some people the condition unfortunately persists.

• #29 Bullous Pemphigoid: What It Is and How to Treat It | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/patient-education/bullous-pemphigoid-what-it-and-how-treat-it

  Bullous pemphigoid can last from 1 to 5 years. If untreated, the blisters and open areas of your skin can cause pain and discomfort. You may also get a severe infection in the open areas of your skin. […] With treatment, bullous pemphigoid often goes away in 1 to 5 years. It’s possible to get it again, but it’s usually less severe.

• #30 Bullous pemphigoid: Diagnosis and treatment

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

  Bullous pemphigoid is a disease that can cause widespread blisters, itching, and rash on your skin, and sometimes even inside your mouth and other areas. […] This disease tends to come and go. You will likely have times when you continue to get new blisters and weeks when you have few if any blisters. Sometimes, this disease goes away without treatment and the person never develops blisters again. […] Medical records show that before treatment was available for bullous pemphigoid, about 20% of patients went into remission after 15 months. This means that the itch, rash, and blisters went away after 15 months without treatment. […] If you have bullous pemphigoid, treatment can reduce the amount of time that it takes for bullous pemphigoid to go into remission. […] For a few patients, remission begins 9 weeks after starting treatment. About 50% of patients go into remission about two years after treatment starts.

• #31 Bullous pemphigoid: Diagnosis and treatment

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

  To stay in remission, you may need to continue treating bullous pemphigoid. Some patients continue to treat it for months to years to keep the disease under control. […] Treatment can help heal your skin so that bullous pemphigoid goes into remission. […] Treatment can also help relieve the itch and pain. […] Most patients follow a treatment plan for six months to five years before the disease goes into long-term remission. Once the disease is in long-term remission, many patients can stop treatment. However, some patients need to continue treatment.

• #32 Bullous Pemphigoid: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/1062391-overview

  Bullous pemphigoid is a chronic, autoimmune, subepidermal, blistering skin disease that rarely involves mucous membranes. If untreated, bullous pemphigoid can persist for months or years, with periods of spontaneous remissions and exacerbations. It can be fatal, particularly in patients who are debilitated. […] Bullous pemphigoid may present with several distinct clinical presentations, as follows: Generalized bullous form – The most common presentation; tense bullae arise on any part of the skin surface, with a predilection for the flexural areas of the skin. Vesicular form – Less common than the generalized bullous type; manifests as groups of small, tense blisters, often on an urticarial or erythematous base. […] Bullous pemphigoid is a chronic inflammatory disease. If untreated, bullous pemphigoid can persist for months or years, with periods of spontaneous remissions and exacerbations. Most patients affected with bullous pemphigoid require therapy for 6-60 months, after which many experience long-term remission of the disease. Some patients, however, have long-standing disease necessitating treatment for years. The lesions typically heal without scarring or milia formation. […] Bullous pemphigoid may be fatal, particularly in patients who are debilitated. The proximal causes of death are infection with sepsis and adverse events associated with treatment. Most of the mortality associated with bullous pemphigoid is secondary to the effects of the medications.

• #33

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6486083/

  Complications related to the disease, such as secondary infection leading to hospitalization and sepsis, or to adverse effects of the medications required for disease control translates into a 3.6-fold higher mortality than age-matched population. […] Risk factors related to increased mortality in BP include older age, neurological disorder (dementia, including Alzheimer’s disease and other types of dementia, and stroke), and increased serum levels of anti-BP180 NC16A IgG. […] A recent systematic review and meta-analysis included 25 studies and demonstrated a 1-year combined mortality rate of 23.5%.

• #34 Pemphigoid: Symptoms, types, and treatment

  https://www.medicalnewstoday.com/articles/318015

  Most forms of pemphigoid cause skin rashes and blistering. People with pemphigoid usually experience symptoms periodically in between times of remission, often for months to years. […] Bullous pemphigoid tends to cause blistering on areas, such as the lower torso, groin, armpits, inner thighs, soles, and palms. […] The condition often presents as extremely itchy patches of raised, irritated skin along with blisters that do not burst readily. Blisters filled with clear or bloody fluid can range in width from a few millimeters to centimeters. […] Most people with bullous pemphigoid experience a flare-up of symptoms followed by periods without symptoms. The condition can persist for years. […] The symptoms sometimes resolve on their own. In older adults or those with weakened immune systems, however, it can cause serious health complications. If blisters burst and become infected, life-threatening blood infections can occur. […] Even with medical treatment, 1-year mortality rates for severe cases of bullous pemphigoid may be as high as 25 to 30 percent. Some research also suggests a link between bullous pemphigoid and the recurrence of existing cancers.

• #35 Bullous pemphigoid – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/diagnosis-treatment/drc-20350419

  Bullous pemphigoid is usually goes away in time. Sores can take weeks to heal, and it’s common for new ones to form. […] Blisters on the feet and hands can make it difficult to walk or to go about daily tasks. The itching may make it hard to sleep. You may need to change your routine until the blisters are under control.

• #36 Factors Influencing the Clinical Course of Bullous Pemphigoid among Geriatric Patients: A Pilot Study

  https://www.mdpi.com/1648-9144/60/10/1701

  The research community agrees that the pathogenesis of pruritus in BP is complex and requires further investigation. It has been demonstrated that for patients with BP, pruritus harms their quality of life. Consequently, targeted treatment is essential to address this significant issue. The results of our study indicated a positive correlation between mean corpuscular hemoglobin concentration (MCHC) levels and the incidence of pruritus, while serum sodium levels were negatively correlated with pruritus risk. […] In our study of patients with BP, we observed that 33.33% of the women and 23.81% of the men had developed neurological diseases (NDs). The most prevalent neurological condition observed in our cohort was dementia, which was diagnosed in three women and two men. In 17.24% of the women and 19.05% of the men, stroke preceded the development of skin lesions. These findings are consistent with previous studies that reported rates of neurological diseases in BP patients ranging from 23% to 73.8%. A significant correlation between the presence of neurological diseases and an elevated risk of erosions in our BP patients was observed, consistent with the pathophysiological mechanisms of BP and the existing literature.

• #37 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.

• #38 Pemphigus and Pemphigoid Signs & Symptoms | Rush

  https://www.rush.edu/conditions/pemphigus-and-pemphigoid

  You develop recurring, unexplained blisters on any part of your body: Pemphigus and pemphigoid blisters can occur on the scalp, face, back, abdomen, arms and legs. They can also occur under the arms, on the genitals and in the mouth, throat or eyes. […] Your blisters are itchy or painful: Pemphigus blisters tend to be painful. They also break open easily, which can cause open sores on the skin. Pemphigoid blisters are often itchy and may occur with an eczema-like rash or hives. These blisters are firmer, meaning they dont break open as easily, and can leave permanent marks on the skin. […] Your blisters make it hard to get through the day: If blisters develop in your eyes, mouth or throat, you may have difficulty seeing, chewing or swallowing. Blisters on your hands or feet can make it hard to work, walk or take care of yourself. And blisters that break easily can leave painful, open sores on your skin. […] Pemphigus usually affects people between 40-60 years of age, while pemphigoid tends to develop after age 60.

• #39 Factors Influencing the Clinical Course of Bullous Pemphigoid among Geriatric Patients: A Pilot Study

  https://www.mdpi.com/1648-9144/60/10/1701

  The analysis of laboratory results in combination with the clinical course of BP showed that red blood cell count, hemoglobin, and hematocrit values were negatively associated with a risk of erosions and erythema, while MCHC values were positively correlated with a risk of associated pruritus. A correlation was found between neurological diseases and an increased risk of erosions. We have shown that age and neurological conditions, including stroke, affect the clinical course of BP. […] The intensity of pruritus was assessed as moderate (with values between 4 and 6 points). In four patients (6%), the main symptoms were pruritic skin and/or erythematous or erosive lesions, while blisters were absent. Studying the relationship between age and the clinical course of BP revealed that there was a much higher incidence of erosions in patients over the age of 85, while patients aged 65–75 and 76–85 were more likely to have blisters.

• #40 Factors Influencing the Clinical Course of Bullous Pemphigoid among Geriatric Patients: A Pilot Study

  https://www.mdpi.com/1648-9144/60/10/1701

  Bullous pemphigoid (BP) is the most common autoimmune blistering disease affecting mainly elderly patients. The disease typically presents as a generalized pruritic bullous skin eruption, with polymorphic lesions being a possibility. However, its typical and most common form involves the eruption of inflammatory skin lesions. These lesions progress from erythema through to urticarial plaques, blisters, and erosions and then to uninflamed, scarless re-epithelizing skin. During the early prodromal phase of BP, the lesions may be non-specific, and bullae or vesicles may be absent. Patients may present with a range of symptoms, including mild to severe pruritus, in isolation or in conjunction with excoriated, eczematous, papular, or urticarial eruptions that may persist for several weeks or even months. When blisters are absent, a diagnosis of the atypical variant of the disease, non-bullous pemphigoid (NBP), is made. NBP is characterized by pruritus and a range of skin manifestations such as urticarial plaques and papules or nodules, which can resemble other pruritic skin disorders.

• #41 Bullous Pemphigoid Pictures, Treatment, Causes, Symptoms

  https://www.medicinenet.com/bullous_pemphigoid/article.htm

  In addition to large, fluid-filled blisters on the skin surface, symptoms of bullous pemphigoid include intense itching and a burning sensation in the skin. […] When the mucous membranes of the mouth are affected, it can cause the following: Pain, Burning, Peeling away of affected inner lining tissues, Sensitivity to acidic foods. […] Eating can be difficult, and involvement in the deeper areas of the throat can cause coughing. Involvement of the inner nose can cause nosebleeds. […] The disease typically worsens and improves over time. It may disappear independently after a few months or persist for years. […] The outlook for bullous pemphigoid is variable. As described above, the symptoms tend to wax and wane. In its most severe form, it can be fatal without treatment, especially if involving the airways and pharynx.

• #42 Childhood bullous pemphigoid

  https://dermnetnz.org/topics/childhood-bullous-pemphigoid

  Childhood bullous pemphigoid is a rare blistering disease affecting children under the age of 18. […] The diagnosis of childhood bullous pemphigoid is based on the following features: Crops of large, tense, fluid-filled blisters (bullae) […] Blisters may be generalised on the skin […] In infants under 1 year of age, blisters tend to occur on hands and feet […] Overall, patients with childhood bullous pemphigoid have a favourable prognosis. The rash eventually clears up and treatment can be withdrawn.

• #43 Systemic lupus erythematosus

  https://diseases.autoimmuneregistry.org/disease_profile.aspx/bullous-pemphigoid.aspx

  Bullous pemphigoid is a rare, autoimmune, chronic skin disorder characterized by blistering. This disorder occurs most frequently in elderly people. Generalized blistering occurs in and under the upper layers of the skin and usually subsides spontaneously within several months or years. In some rare cases of Bullous pemphigoid, complications such as pneumonia may develop. […] Symptoms: Blisters, Hives, Difficulty swallowing, Blisters on eyes, Large blisters on lower abdomen, groin, upper thighs and arms that don’t easily rupture when touched, Wart-like plaques on areas of the skin, such as the armpit, neck, groin, and breasts, Blisters in infants, Flare/remission pattern to symptoms, Blister inside mouth, In childhood-onset bullous pemphigoid associated with vaccination, the bullous lesions predominantly affect the palms, soles, and face.

• #44 Bullous pemphigoid as a diagnostic and therapeutic challenge with a wide spectrum of dermatological symptoms

  https://www.termedia.pl/Bullous-pemphigoid-as-a-diagnostic-and-therapeutic-challenge-with-a-wide-spectrum-of-dermatological-symptoms,56,55448,1,1.html

  After 1.5 years from the onset of the initial symptoms, new blisters appeared on the upper extremities, accompanied by erosions and erythematous changes on the extensor surface of the left forearm, accompanied by itching. Laryngological examination revealed blistering lesions in the area of the epiglottic folds. Finally, the lesions also involved the vulva. Therefore, systemic GCS therapy was initiated while continuing topical treatment, achieving complete remission of the disease. […] A paucisymptomatic form limited to mucous membranes in BP can be challenging to diagnose, thus requiring precise differential diagnosis. Only the histopathological image and the presence of specific antibodies confirm the diagnosis. […] A specific feature of BP is the occurrence of lesions induced by physical factors (such as trauma, surgical procedures, thermal burns). In our patient, blisters appeared under a forearm cast. The patient experienced a scalding water burn, after which blisters developed that did not resolve despite wound healing, but improved only after GCS treatment was initiated.

• #45 Bullous pemphigoid as a diagnostic and therapeutic challenge with a wide spectrum of dermatological symptoms

  https://www.termedia.pl/Bullous-pemphigoid-as-a-diagnostic-and-therapeutic-challenge-with-a-wide-spectrum-of-dermatological-symptoms,56,55448,1,1.html

  The initial symptoms of the disease usually manifest within a year, although they can occur even several years after exposure to the physical factor. […] Drug-induced bullous disorders can be caused by antibiotics, -blockers, angiotensin-converting enzyme (ACE) inhibitors, and neuroleptics (such as haloperidol, risperidone, biperiden). The more medications patients take, the more difficult it is to determine that one triggering BP. […] BP can also be triggered by vaccination (e.g., influenza, tetanus), infections, and in association with haematological malignancies, renal or laryngeal cancer. It has been observed that individuals with multiple sclerosis, schizophrenia, dementia, stroke, delusional disorders, personality disorders, and Parkinson’s disease may exhibit BP symptoms. The risk of developing BP is three times higher in patients with dementia and Parkinson’s disease, and twice as high in patients with a history of stroke or epilepsy.

• #46 Autoimmune and blistering diseases: What you need to know about bullous pemphigoid, pemphigus and mucous membrane pemphigoid | Nebraska Medicine Omaha, NE

  https://www.nebraskamed.com/health/conditions-and-services/dermatology/autoimmune-and-blistering-diseases-what-you-need-to-know

  Bullous pemphigoid is the most common autoimmune blistering disease, with fewer than 20,000 diagnosed among the U.S. population each year. […] This skin disease is more common among older adults in their 60s to 80s. […] Common symptoms include: itchy skin that may develop weeks or months before blisters form, large, hard, fluid-filled blisters along the creases or folds in the skin often located in the abdomen, upper thighs or armpits, red or dark colored skin around the blisters, small blisters in the mouth or other mucous membranes. […] Bullous pemphigoid can sometimes be triggered by certain blood pressure meds, certain antibiotics or even specific cancer treatments. […] The blisters will go away on its own in a few months. But for others, they may hang on for five years or more. Treatment usually involves anti-inflammatories and immunosuppression drugs that put the disease into remission.

• #47 Bullous pemphigoid as a diagnostic and therapeutic challenge with a wide spectrum of dermatological symptoms

  https://www.termedia.pl/Bullous-pemphigoid-as-a-diagnostic-and-therapeutic-challenge-with-a-wide-spectrum-of-dermatological-symptoms,56,55448,1,1.html

  Bullous pemphigoid (BP) is considered the most common autoimmune blistering dermatosis in Europe and in the USA. It is a chronic inflammatory skin disease that affects between 190300 individuals per million annually. It occurs more frequently in men than in women, predominantly in individuals over 80 years old. This dermatosis is characterized by the presence of well-tense blisters in the area of erythematous-oedematous eruptions, as well as in unaffected skin. In some patients, the lesions may also appear on mucous membranes (20% of cases). Untreated BP can persist for months or years, with periods of remission and exacerbation. Although this dermatosis typically manifests on the skin, in our case, we present a patient in whom the disease primarily manifests in the oral cavity (on the soft and hard palate).

• #48 Dupixent Meets Endpoints in Phase 2/3 Bullous Pemphigoid Study – IPPF

  https://www.pemphigus.org/dupixent-meets-endpoints-in-phase-2-3-bullous-pemphigoid-study/

  BP, a chronic and relapsing disease, is characterized by intense itch and blisters, reddening of the skin, and painful chronic lesions. The blisters and rash can form over much of the body and cause the skin to bleed and crust, resulting in patients being more prone to infection and affecting their daily functioning. […] The itchy blisters caused by bullous pemphigoid can be so intense they are debilitating, especially for elderly patients, said Dietmar Berger, M.D., Ph.D., Chief Medical Officer, Global Head of Development at Sanofi. […] Bullous pemphigoid is a debilitating skin disease with a high mortality rate due to infection, said George D. Yancopoulos, M.D., Ph.D., Board co-Chair, President, and Chief Scientific Officer at Regeneron. Dupixent is the first medication to show significant and robust impacts in this patient population.

• #49 Press Release: Dupixent is the first and only biologic to achieve significant improvements in disease remission and symptoms in bullous pemphigoid positive pivotal study

  https://www.sanofi.com/en/media-room/press-releases/2024/2024-09-11-05-00-00-2944237

  Dupixent is the first and only biologic to achieve significant improvements in disease remission and symptoms in bullous pemphigoid positive pivotal study. […] BP, a chronic and relapsing disease, is characterized by intense itch and blisters, reddening of the skin, and painful chronic lesions. The blisters and rash can form over much of the body and cause the skin to bleed and crust, resulting in patients being more prone to infection and affecting their daily functioning. […] The itchy blisters caused by bullous pemphigoid can be so intense they are debilitating, especially for elderly patients. […] Bullous pemphigoid is a debilitating skin disease with a high mortality rate due to infection.

• #50 Bullous Pemphigoid: Understanding and Managing this Rare Skin Disorder | Maggie Yu MD, IFMCP

  https://drmaggieyu.com/blog/bullous-pemphigoid-a-detailed-guide-to-understanding-and-managing-this-rare-skin-disorder/

  Bullous pemphigoid, a disease examined by dermatologists, is a skin disorder that primarily presents with blisters and hives. This condition involves the immune system producing autoantibodies. These bullous lesions, also known as skin disorders, are tense blisters filled with fluid, often requiring a dermatologist’s expertise in disease and wound care. […] The widespread blisters, often a disease symptom, typically appear on the patient’s abdomen, arms, and legs, according to the dermatologist’s training. Sometimes, they can even pop up inside your mouth. […] Early detection is super important for effective treatment of bp, also known as bullous pemphigoid. The sooner bp is found, the better chance you have of managing it successfully. […] If left undiagnosed or untreated, bullous pemphigoid, often referred to as BP, can cause some serious complications like infection and even sepsis (a life-threatening response to infection).

• #51 Bullous Pemphigoid: Clinical Practice Guidelines | Actas Dermo-Sifiliográficas

  https://actasdermo.org/en-bullous-pemphigoid-clinical-practice-guidelines-articulo-S1578219014000778

  Bullous pemphigoid (BP) is an autoimmune blistering disease in which autoantibodies are directed against components of the basement membrane. Most of these antibodies belong to the immunoglobulin G class and bind principally to 2 hemidesmosomal proteins: the 180-kD antigen (BP180) and the 230-kD antigen (BP230). It is the most common blistering disease in the adult population in developed countries, with an estimated incidence in Spain of 0.2 to 3 cases per 100,000 inhabitants per year. The disease primarily affects older people, although it can also occur in young people and even in children. […] Clinically, BP is characterized by the appearance of highly pruritic urticaria-like or eczematous lesions on which tense blisters may develop after a variable period of time. Some patients only develop intense itching, and the only lesions visible in these cases are excoriations. Pemphigoid should therefore be considered in the differential diagnosis of chronic itching in an elderly patient. Patients initially develop vesicles, but these may progress to large blisters that are typically filled with a clear fluid or blood. They are located predominantly on the trunk and the flexor surface of the extremities, and rarely affect the head or neck. The Nikolsky sign is negative, although a positive result does not rule out a diagnosis of BP. In our practice, we have seen some cases of BP with a positive Nikolsky sign.

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