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. […] While most bullous pemphigoid cases are due to autoantibodies against proteins arranged at the dermal-epidermal junction, some cases of bullous pemphigoid are caused by systemic medications. Drug-induced bullous pemphigoid occurs up to 3 months after medication initiation and is typically noted in a younger subset of patients. The drugs reported in the literature to cause bullous pemphigoid-like eruptions include diuretics such as furosemide and spironolactone, NSAIDs, amoxicillin, PD-1/PD-L1 inhibitors, gliptins, and TNF-alpha inhibitors.

• #2 Bullous Pemphigoid & Pemphigus Vulgaris- Dermatology Advisor

  https://www.dermatologyadvisor.com/ddi/bullous-pemphigoid-pemphigus-vulgaris/

  Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease. The mean age of presentation is between 75 and 85 years, and the risk of developing BP increases with age. BP is an autoimmune disorder in which immunoglobulin G (IgG) autoantibodies attack BP180 (BPAG2, type XVIII collagen) and BP230 (BPAG1-e) antigens found on hemidesmosomes, which are responsible for the tight barrier between the epidermis and dermis. […] Many triggers are associated with BP, some of which include medications and viral infections. Certain drugs, such as loop diuretics and neuroleptics, are thought to alter the immune response to specific antigens or, conversely, modify the antigenic properties of the epidermal basement membrane leading to the development of BP. Infections such as human herpesvirus, hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii have also been associated with the development of BP. Other triggers linked to the development of this disease include trauma, burns, radiotherapy, ultraviolent radiation, various autoimmune disorders, psoriasis, and certain neurologic disorders.

• #3 Orphanet: Bullous pemphigoid

  https://www.orpha.net/en/disease/detail/703

  Bullous pemphigoid (BP) is immunologically characterized by the production of autoantibodies directed against two structural proteins found in the dermal-epidermal junction and ensuring dermal-epidermal cohesion: BP antigen 1 (BPAG1 or AgBP230), and BP antigen 2 (BPAG2, AgBP180 or collagen XVII). The binding between the autoantibodies and these proteins leads to the separation between the dermis and the epidermis and the formation of blisters. Some drugs are associated with the onset of BP (diuretics, antiarrhythmics, neuroleptics, gliptins, immunotherapies). […] BP predominantly affects the elderly with an average age of 80 years and is significantly associated with neurological disorders. Some cases have also been described in children and young adults.

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

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

  Bullous pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells). The target is the protein BP180 (also called Type XVII collagen), or less frequently BP230 (a plakin). These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. […] The binding of the autoantibodies to the proteins and release of cytokines from the T cells lead to complement activation, recruitment of neutrophils (acute inflammatory cells) and the release of proteolytic enzymes. These destroy the hemidesmosomes and cause the formation of subepidermal blisters. […] The association of neurological diseases with bullous pemphigoid is thought to relate to the presence of collagen XVII in the central nervous system and skin hemidesmosomes. […] A rare variant of pemphigoid with IgG antibodies directed against a 200 kDa DEJ protein has been described there is a strong association with psoriasis (laminin gamma 1).

• #5 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. Bullous pemphigoid is characterized by the presence of immunoglobulin G (IgG) autoantibodies specific for the hemidesmosomal bullous pemphigoid antigens (BPAgs) BP230 (BPAg1) and BP180 (BPAg2). […] The precise role of bullous pemphigoid antigens (BPAgs) in the pathogenesis of bullous pemphigoid is not completely clear. BPAg1 (BP230) is an intracellular component of the hemidesmosome; BPAg2 (BP180, type XVII collagen) is a transmembranous protein with a collagenous extracellular domain. […] Although BPAg2 has been identified as the major antigen involved with bullous pemphigoid disease development, autoantibodies against alpha 6 integrin and laminin-5, two other components of skin basement membrane, have been identified in human patients affected by bullous pemphigoid.

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

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

  Bullous pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells). The target is the protein BP180 (also called Type XVII collagen), or less frequently BP230 (a plakin). These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. […] The binding of the autoantibodies to the proteins and release of cytokines from the T cells lead to complement activation, recruitment of neutrophils (acute inflammatory cells) and the release of proteolytic enzymes. These destroy the hemidesmosomes and cause the formation of subepidermal blisters. […] The association of neurological diseases with bullous pemphigoid is thought to relate to the presence of collagen XVII in the central nervous system and skin hemidesmosomes. […] A rare variant of pemphigoid with IgG antibodies directed against a 200 kDa DEJ protein has been described there is a strong association with psoriasis (laminin gamma 1).

• #7 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. It is classified as a type II hypersensitivity reaction, which involves formation of anti-hemidesmosome antibodies, causing a loss of keratinocytes to basement membrane adhesion. […] In most cases of bullous pemphigoid, no clear precipitating factors are identified. Potential precipitating events that have been reported include exposure to ultraviolet light and radiation therapy. Onset of pemphigoid has also been associated with certain drugs, including furosemide, nonsteroidal anti-inflammatory agents, DPP-4 inhibitors, captopril, penicillamine, and antibiotics. […] The bullae are formed by an immune reaction, initiated by the formation of IgG autoantibodies targeting dystonin, also called bullous pemphigoid antigen 1, and/or type XVII collagen, also called bullous pemphigoid antigen 2, which is a component of hemidesmosomes.

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

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

  Bullous pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells). The target is the protein BP180 (also called Type XVII collagen), or less frequently BP230 (a plakin). These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. […] The binding of the autoantibodies to the proteins and release of cytokines from the T cells lead to complement activation, recruitment of neutrophils (acute inflammatory cells) and the release of proteolytic enzymes. These destroy the hemidesmosomes and cause the formation of subepidermal blisters. […] The association of neurological diseases with bullous pemphigoid is thought to relate to the presence of collagen XVII in the central nervous system and skin hemidesmosomes. […] A rare variant of pemphigoid with IgG antibodies directed against a 200 kDa DEJ protein has been described there is a strong association with psoriasis (laminin gamma 1).

• #9 Bullous Pemphigoid – الجمعية السعودية لأمراض وجراحة الجلد

  https://ssdds.org/en/5396/

  Bullous pemphigoid occurs when IgG and IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells) attack the basement membrane of the epidermis. The target protein is BP180, also known as Type XVII collagen, or occasionally BP230, which is a plakin. The proteins are located inside the NC16A domain of collagen XVII. They are linked to the hemidesmosomes, which are structures responsible for the adhesion of epidermal keratinocyte cells to the dermis, creating a water-resistant barrier. […] The autoantibodies attaching to the proteins and subsequent release of cytokines from the T cells result in complement activation, recruitment of neutrophils (acute inflammatory cells), and the release of proteolytic enzymes. These disrupt the hemidesmosomes and induce the development of blisters under the epidermis.

• #10

  https://link.springer.com/article/10.1007/s12325-024-02992-w

  Bullous pemphigoid (BP) is an autoimmune blistering disease that most often affects elderly individuals and has a significant negative impact on quality of life. The disease is characterized primarily by autoantibodies to hemidesmosomal proteins BP180 and/or BP230, and an inflammatory reaction with notable features of type 2 inflammation, including elevated serum IgE, increased numbers of eosinophils in lesions and peripheral blood, and elevated expression of type 2 cytokines and chemokines in skin lesions. […] BP pathophysiology is not completely understood but is characterized primarily by the production of immunoglobin G (IgG) autoantibodies directed against hemidesmosomal anchoring proteins BP antigen 180 (BP180) and/or BP antigen 230 (BP230). […] Several lines of evidence also suggest a prominent type 2 inflammatory response in BP. Type 2 inflammation predominantly involves the activation of group 2 innate lymphoid cells, T helper type 2 cells, eosinophils, and inflammatory cytokines such as interleukin (IL)-4, IL-5, and IL-13.

• #11

  https://link.springer.com/article/10.1007/s12325-024-02992-w

  In patients with BP, immunoglobin E (IgE) and eosinophils were elevated in both peripheral blood and skin lesions, and levels of inflammatory cytokines IL-4, IL-5, and IL-13 were also increased in skin lesions. […] Elevated serum levels of IgE are also seen in patients with BP, with anti-BP180 and anti-BP230 IgE autoantibodies playing an important role. […] Recent research also suggests that IgE and BP180 form immune complexes in BP skin, which may activate mast cells and eosinophils through the high-affinity IgE receptor FcRI. […] Several lines of evidence point to an important role of type 2 inflammatory cytokines and chemokines in BP pathophysiology. […] In BP lesions, levels of type 2 inflammatory cytokines IL-4, IL-5, and IL-13 are elevated, as are levels of chemokines CCL11 (also called eotaxin 1), CCL26 (eotaxin 3), CCL13 (monocyte chemoattractant protein 4, or MCP-4), and CCL17 (thymus- and activation-regulated chemokine, or TARC).

• #12 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. […] The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane.

• #13 Bullous Pemphigoid Treatment Chatsworth Dermatologist

  https://chatsworthdermatology.com/medical-dermatology/bullous-pemphigoid/

  Bullous pemphigoid is caused by a problem with the immune system, which attacks the skin instead of germs. The immune system produces antibodies that damage the basement membrane zone, a thin layer of tissue below the outer layer of skin. This leads to inflammation and blisters on the skin. The reason for this abnormal immune response is unknown, although it may be triggered by certain medications, light and radiation therapy, medical conditions, or skin damage. […] The risk factors for developing bullous pemphigoid include old age, genetic predisposition, neurological diseases, certain medications, light and radiation therapy, and internal malignancy. Old age is the most common risk factor, as most cases occur in people over 60 years old. Genetic predisposition may play a role in some cases, as some people have a variation in the HLA-DQB1 gene that increases their susceptibility to bullous pemphigoid. Neurological diseases such as dementia, Parkinson’s disease, stroke, epilepsy, and multiple sclerosis may trigger or worsen bullous pemphigoid. Certain medications such as etanercept, sulfasalazine, furosemide, and penicillin may induce or exacerbate bullous pemphigoid.

• #14 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. […] The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane.

• #15 Bullous Pemphigoid Treatment Chatsworth Dermatologist

  https://chatsworthdermatology.com/medical-dermatology/bullous-pemphigoid/

  Bullous pemphigoid is caused by a problem with the immune system, which attacks the skin instead of germs. The immune system produces antibodies that damage the basement membrane zone, a thin layer of tissue below the outer layer of skin. This leads to inflammation and blisters on the skin. The reason for this abnormal immune response is unknown, although it may be triggered by certain medications, light and radiation therapy, medical conditions, or skin damage. […] The risk factors for developing bullous pemphigoid include old age, genetic predisposition, neurological diseases, certain medications, light and radiation therapy, and internal malignancy. Old age is the most common risk factor, as most cases occur in people over 60 years old. Genetic predisposition may play a role in some cases, as some people have a variation in the HLA-DQB1 gene that increases their susceptibility to bullous pemphigoid. Neurological diseases such as dementia, Parkinson’s disease, stroke, epilepsy, and multiple sclerosis may trigger or worsen bullous pemphigoid. Certain medications such as etanercept, sulfasalazine, furosemide, and penicillin may induce or exacerbate bullous pemphigoid.

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

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

  Genetic and environmental factors may play a role. […] Triggers may induce an autoimmune reaction by mimicking molecular sequences in the epidermal basement membrane (molecular mimicry, as with drugs and possibly infections), by exposing or altering normally tolerated host antigens (as with physical triggers and certain disorders), or by other mechanisms. […] Certain central nervous system (CNS) and psychiatric disorders may precede bullous pemphigoid, especially multiple sclerosis and schizophrenia, but also dementias, intracranial bleeds, stroke, delusional and personality disorders, and Parkinson disease. To a lesser degree, these disorders may be preceded by bullous pemphigoid. Hypothesized shared causes include a cross-reactive immune response between neural and cutaneous antigens (BPAg1 is expressed in the CNS), as well as triggering by certain drugs used to treat the CNS disorders (eg, phenothiazine antipsychotics, spironolactone); however, a mechanism of triggering by drugs is not understood.

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

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

  Bullous pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells). The target is the protein BP180 (also called Type XVII collagen), or less frequently BP230 (a plakin). These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. […] The binding of the autoantibodies to the proteins and release of cytokines from the T cells lead to complement activation, recruitment of neutrophils (acute inflammatory cells) and the release of proteolytic enzymes. These destroy the hemidesmosomes and cause the formation of subepidermal blisters. […] The association of neurological diseases with bullous pemphigoid is thought to relate to the presence of collagen XVII in the central nervous system and skin hemidesmosomes. […] A rare variant of pemphigoid with IgG antibodies directed against a 200 kDa DEJ protein has been described there is a strong association with psoriasis (laminin gamma 1).

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

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

  Genetic and environmental factors may play a role. […] Triggers may induce an autoimmune reaction by mimicking molecular sequences in the epidermal basement membrane (molecular mimicry, as with drugs and possibly infections), by exposing or altering normally tolerated host antigens (as with physical triggers and certain disorders), or by other mechanisms. […] Certain central nervous system (CNS) and psychiatric disorders may precede bullous pemphigoid, especially multiple sclerosis and schizophrenia, but also dementias, intracranial bleeds, stroke, delusional and personality disorders, and Parkinson disease. To a lesser degree, these disorders may be preceded by bullous pemphigoid. Hypothesized shared causes include a cross-reactive immune response between neural and cutaneous antigens (BPAg1 is expressed in the CNS), as well as triggering by certain drugs used to treat the CNS disorders (eg, phenothiazine antipsychotics, spironolactone); however, a mechanism of triggering by drugs is not understood.

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

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

  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. […] People who get these diseases are genetically predisposed, says Dr. Tomayko. They could have a difference in their immune responses that make them a little more prone to getting these diseases. Exposures throughout their lives and age-related changes in the immune system could also put them at a higher risk, though we dont know the triggers. In short, its a combination of genes and environment that were still getting a handle on. […] Bullous pemphigoid is more common in people with pre-existing neurological diseases such as Parkinsons disease or dementia. The link between these two kinds of disorders is an active area of research here at Yale Medicine, Dr. Tomayko says. Our working hypothesis is that because people who have Parkinsons and dementia have elevated levels of antibodies against collagen proteins, their immune systems are primed to attack the skin.

• #20 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. […] The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane.

• #21 Bullous Pemphigoid

  https://fpnotebook.com/Derm/Blister/BlsPmphgd.htm

  Idiopathic (85% of cases) […] Specific trigger (15% of cases) […] Radiation Therapy […] Burn Injury […] Immunization […] Surgery […] Trauma […] Medications (esp. Furosemide)

• #22 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. […] The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

• #24 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

• #25 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

• #26 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

• #27 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

• #28 Bullous Pemphigoid: Causes, Symptoms & Treatment

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

  Bullous pemphigoid is an autoimmune disease. Certain medications may trigger bullous pemphigoid, but healthcare providers aren’t sure exactly what causes it. […] Bullous pemphigoid is an autoimmune disease. Healthcare providers and medical researchers aren’t sure why your immune system responds this way. […] Certain medications may trigger bullous pemphigoid, including the diabetes medication alogliptin. Ultraviolet (UV) light and ionizing radiation may also trigger or aggravate bullous pemphigoid.

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

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

  Dipeptidyl peptidase 4 (DPP-4) inhibitors have been associated with a risk of developing bullous pemphigoid. […] Environmental factors have been associated with bullous pemphigoid, including daily consumption of green or herbal tea, fish oil, calcium supplements, multivitamins, and glucosamine. Other triggers associated with bullous pemphigoid include use of lime-containing household cleaning products and high levels of mental stress.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

• #31 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

• #32 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. […] While most bullous pemphigoid cases are due to autoantibodies against proteins arranged at the dermal-epidermal junction, some cases of bullous pemphigoid are caused by systemic medications. Drug-induced bullous pemphigoid occurs up to 3 months after medication initiation and is typically noted in a younger subset of patients. The drugs reported in the literature to cause bullous pemphigoid-like eruptions include diuretics such as furosemide and spironolactone, NSAIDs, amoxicillin, PD-1/PD-L1 inhibitors, gliptins, and TNF-alpha inhibitors.

• #33 Bullous Pemphigoid | Dermatologist In Brevard, NC | Highlands Dermatology

  https://www.highlandsdermatology.com/articles/aad_education_library/920190-bullous-pemphigoid/

  Bullous pemphigoid is a rare, chronic, autoimmune blistering disorder that causes large, fluid-filled blisters on the skin. […] The cause is unknown, but it is thought to be related to an overactive immune system. When your immune system attacks a thin layer of skin called the basement membrane, it causes blisters to form. […] The painful blisters occur as a result of an immune system malfunction. In people with bullous pemphigoid, the immune system attacks the basement membrane layer of the skin. This results in blistering and inflammation. […] The exact cause of this autoimmune response is unknown. However, certain factors may play a role, including: […] UV radiation therapies for skin conditions and radiation treatment for cancer can trigger bullous pemphigoid. […] As you get older, the likelihood of developing bullous pemphigoid increases. […] Medications that may cause bullous pemphigoid to include Enbrel, Lasix, Azulfidine, and penicillin. […] If you suffer from a disorder such as psoriasis, rheumatoid arthritis, ulcerative colitis, or multiple sclerosis (MS), these conditions could play a role in developing bullous pemphigoid.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

• #35 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. […] While most bullous pemphigoid cases are due to autoantibodies against proteins arranged at the dermal-epidermal junction, some cases of bullous pemphigoid are caused by systemic medications. Drug-induced bullous pemphigoid occurs up to 3 months after medication initiation and is typically noted in a younger subset of patients. The drugs reported in the literature to cause bullous pemphigoid-like eruptions include diuretics such as furosemide and spironolactone, NSAIDs, amoxicillin, PD-1/PD-L1 inhibitors, gliptins, and TNF-alpha inhibitors.

• #36 Bullous Pemphigoid – MD Searchlight

  https://mdsearchlight.com/skin-problems-and-treatments/bullous-pemphigoid/

  Most cases of bullous pemphigoid, a skin condition that causes blisters, often occur due to an immune response against proteins at where the skin layers meet. However, there are instances where this condition is caused by certain medications. This so-called drug-induced bullous pemphigoid can happen up to three months after starting the medicine and is typically seen in younger patients. […] Several drugs have been reported to trigger this blistering skin reaction. These include some water pills (like furosemide and spironolactone), pain relievers called NSAIDs, the antibiotic amoxicillin, medications that inhibit PD-1/PD-L1 (used in cancer treatment), gliptins (used for diabetes), and TNF-alpha inhibitors (used for certain autoimmune conditions).

• #37 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  A contributing role in inducing BP has been suggested for infections, in particular human herpes virus (HHV) infections (cytomegalovirus, Epstein-Barr virus, and HHV-6), but also hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii. Unlike pemphigus, no dietary triggers have been suspected of being involved in the induction of BP. In all patients who have a diagnosis of BP, an environmental agent as a potential cause should always be considered, because the prompt discontinuation of it might result in rapid improvement or even cure of the disease.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

• #39 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

• #40 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. It is classified as a type II hypersensitivity reaction, which involves formation of anti-hemidesmosome antibodies, causing a loss of keratinocytes to basement membrane adhesion. […] In most cases of bullous pemphigoid, no clear precipitating factors are identified. Potential precipitating events that have been reported include exposure to ultraviolet light and radiation therapy. Onset of pemphigoid has also been associated with certain drugs, including furosemide, nonsteroidal anti-inflammatory agents, DPP-4 inhibitors, captopril, penicillamine, and antibiotics. […] The bullae are formed by an immune reaction, initiated by the formation of IgG autoantibodies targeting dystonin, also called bullous pemphigoid antigen 1, and/or type XVII collagen, also called bullous pemphigoid antigen 2, which is a component of hemidesmosomes.

• #41 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

• #43 Bullous pemphigoid

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

  Bullous pemphigoid is caused by a problem with the immune system, the body’s defence against infection. Instead of attacking germs, it attacks and damages the skin. […] It’s not always clear why this happens, although certain things may trigger it. […] These include: certain medicines, sunburn, surgery, burns.

• #44 Bullous Pemphigoid: Trigger and Predisposing Factors

  https://www.mdpi.com/2218-273X/10/10/1432

  Bullous pemphigoid (BP) is the most frequent autoimmune subepidermal blistering disease provoked by autoantibodies directed against two hemidesmosomal proteins: BP180 and BP230. Its pathogenesis depends on the interaction between predisposing factors, such as human leukocyte antigen (HLA) genes, comorbidities, aging, and trigger factors. Several trigger factors, such as drugs, thermal or electrical burns, surgical procedures, trauma, ultraviolet irradiation, radiotherapy, chemical preparations, transplants, and infections may induce or exacerbate BP disease. […] The etiopathogenesis of BP is largely unknown, but in several cases the occurrence or exacerbation of the disease has been reported in association with a specific “trigger factor”, such as drugs, physical factors, vaccines, infections, transplantations, and others. […] The aim of the present review is to highlight the trigger and predisposing factors underlying the onset of BP.

• #45 Bullous pemphigoid

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

  Bullous pemphigoid is caused by a problem with the immune system, the body’s defence against infection. Instead of attacking germs, it attacks and damages the skin. […] It’s not always clear why this happens, although certain things may trigger it. […] These include: certain medicines, sunburn, surgery, burns.

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

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

  Genetic and environmental factors may play a role. […] Triggers may induce an autoimmune reaction by mimicking molecular sequences in the epidermal basement membrane (molecular mimicry, as with drugs and possibly infections), by exposing or altering normally tolerated host antigens (as with physical triggers and certain disorders), or by other mechanisms. […] Certain central nervous system (CNS) and psychiatric disorders may precede bullous pemphigoid, especially multiple sclerosis and schizophrenia, but also dementias, intracranial bleeds, stroke, delusional and personality disorders, and Parkinson disease. To a lesser degree, these disorders may be preceded by bullous pemphigoid. Hypothesized shared causes include a cross-reactive immune response between neural and cutaneous antigens (BPAg1 is expressed in the CNS), as well as triggering by certain drugs used to treat the CNS disorders (eg, phenothiazine antipsychotics, spironolactone); however, a mechanism of triggering by drugs is not understood.

• #47 Bullous Pemphigoid | IntechOpen

  https://www.intechopen.com/chapters/59076

  Bullous pemphigoid (BP) is a chronic, acquired, autoimmune bullous disease characterized by subepidermal bullae. […] Autoantibodies against hemidesmosomal proteins BP230 (BPAG1) and BP180 (BPAG2 or type XVII collagen) are blamed for the pathogenesis. […] Autoantibodies against hemidesmosomal proteins BP230 (BPAG1) and BP180 (BPAG2 or type XVII collagen) are blamed for the pathogenesis. […] It has been shown that furosemide, psoralen, ibuprofen, galantamine, hydrobromide, ACEi, spironolactone, penicillin, levofloxacin, metronidazole may lead to BP. […] Adalimumab and etanercept (TNF-alpha blockers) associated BP cases have also been reported. […] Another cause blamed in the etiology is vaccinations. BP may develop after 1 day-4 weeks of vaccination. […] In addition, trauma, radiotherapy and UVB exposure may cause bullous lesions by uncovering BP antigens.

• #48 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  A contributing role in inducing BP has been suggested for infections, in particular human herpes virus (HHV) infections (cytomegalovirus, Epstein-Barr virus, and HHV-6), but also hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii. Unlike pemphigus, no dietary triggers have been suspected of being involved in the induction of BP. In all patients who have a diagnosis of BP, an environmental agent as a potential cause should always be considered, because the prompt discontinuation of it might result in rapid improvement or even cure of the disease.

• #49 Bullous Pemphigoid & Pemphigus Vulgaris- Dermatology Advisor

  https://www.dermatologyadvisor.com/ddi/bullous-pemphigoid-pemphigus-vulgaris/

  Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease. The mean age of presentation is between 75 and 85 years, and the risk of developing BP increases with age. BP is an autoimmune disorder in which immunoglobulin G (IgG) autoantibodies attack BP180 (BPAG2, type XVIII collagen) and BP230 (BPAG1-e) antigens found on hemidesmosomes, which are responsible for the tight barrier between the epidermis and dermis. […] Many triggers are associated with BP, some of which include medications and viral infections. Certain drugs, such as loop diuretics and neuroleptics, are thought to alter the immune response to specific antigens or, conversely, modify the antigenic properties of the epidermal basement membrane leading to the development of BP. Infections such as human herpesvirus, hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii have also been associated with the development of BP. Other triggers linked to the development of this disease include trauma, burns, radiotherapy, ultraviolent radiation, various autoimmune disorders, psoriasis, and certain neurologic disorders.

• #50

  https://journals.lww.com/md-journal/fulltext/2018/04130/bullous_pemphigoid_associated_with_chronic.55.aspx

  Bullous pemphigoid is a type of acute or chronic autoimmune disease that involves subepidermal skin lesions with bulla formation. […] Although viral infections, such as, human herpes virus (HHV), human immunodeficiency virus, cytomegalovirus, EpsteinBarr virus, HHV-6, hepatitis B virus (HBV), and hepatitis C virus (HCV), are known factors of bullous pemphigoid, HCV infection has only been rarely associated factor, especially in HBV endemic area. […] Although viral hepatitis infections such as hepatitis B virus (HBV) and hepatitis C virus (HCV) are also known to predispose the disease, HCV infection is only very rarely associated, especially in HBV endemic areas. […] The known common potential causes of bullous pemphigoid are ultraviolet light, radiation therapy, and nonsteroidal anti-inflammatory drugs, and although uncommon, viral infections, such as, HHV, HIV, cytomegalovirus, EpsteinBarr virus, HHV-6, HBV, and HCV have also been associated with the disease.

• #51

  https://journals.lww.com/md-journal/fulltext/2018/04130/bullous_pemphigoid_associated_with_chronic.55.aspx

  However, relations between these viral infections and bullous pemphigoid remain the topic of debate, and to our knowledge, no known report has yet linked bullous pemphigoid and chronic HCV infection in a HBV endemic area. […] The role of viral hepatitis C in its pathogenesis remains to be determined. […] In a previous study, the prevalence of HCV antibody was found to be higher in patients with bullous pemphigoid than in healthy control. […] In addition, it has been reported that HCV is associated with various skin diseases, such as, pemphigus vulgaris, cutaneous vasculitis, porphyria cutanea tarda, and lichen planus. […] Given that the presence of HCV antibody need not be linked to current or past infection, efforts to diagnose the current HCV infection are required. […] In this case, HCV RNA and HCV genotype were all detected, which suggests current chronic HCV infection may be directly associated with bullous pemphigoid beyond the simple presence of antibody. […] In conclusion, we report a rare case of bullous pemphigoid associated with chronic HCV infection in a HBV endemic area. HCV should be considered in the differential diagnosis of factors that precipitate bullous pemphigoid even in HBV endemic areas.

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

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

  Genetic and environmental factors may play a role. […] Triggers may induce an autoimmune reaction by mimicking molecular sequences in the epidermal basement membrane (molecular mimicry, as with drugs and possibly infections), by exposing or altering normally tolerated host antigens (as with physical triggers and certain disorders), or by other mechanisms. […] Certain central nervous system (CNS) and psychiatric disorders may precede bullous pemphigoid, especially multiple sclerosis and schizophrenia, but also dementias, intracranial bleeds, stroke, delusional and personality disorders, and Parkinson disease. To a lesser degree, these disorders may be preceded by bullous pemphigoid. Hypothesized shared causes include a cross-reactive immune response between neural and cutaneous antigens (BPAg1 is expressed in the CNS), as well as triggering by certain drugs used to treat the CNS disorders (eg, phenothiazine antipsychotics, spironolactone); however, a mechanism of triggering by drugs is not understood.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

• #54 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

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

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

  Bullous pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells). The target is the protein BP180 (also called Type XVII collagen), or less frequently BP230 (a plakin). These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. […] The binding of the autoantibodies to the proteins and release of cytokines from the T cells lead to complement activation, recruitment of neutrophils (acute inflammatory cells) and the release of proteolytic enzymes. These destroy the hemidesmosomes and cause the formation of subepidermal blisters. […] The association of neurological diseases with bullous pemphigoid is thought to relate to the presence of collagen XVII in the central nervous system and skin hemidesmosomes. […] A rare variant of pemphigoid with IgG antibodies directed against a 200 kDa DEJ protein has been described there is a strong association with psoriasis (laminin gamma 1).

• #56 Bullous pemphigoid | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/bullous-pemphigoid?content_id=CON-20154767

  Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines. […] The cause of this problem is not well understood. In some cases, the condition is brought on by: […] Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab. […] Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition. […] Psoriasis, lichen planus, dementia, Parkinson’s disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

• #57 Bullous pemphigoid

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

  Bullous pemphigoid is an immunobullous condition. The immunobullous conditions are characterised by pathogenic autoantibodies directed at target antigens whose function is either cell-to-cell adhesion within the epidermis or adhesion of stratified squamous epithelium to the dermis or mesencyme. The target antigens involved are components of desmosomes or the functional unit of the basement membrane zone known as the adhesion complex. […] In bullous pemphigoid the autoantibodies, chiefly IgG, are directed to the basement membrane zone, particularly the bullous pemphigoid antigens BP180 and BP230. […] Drugs – although there are no well-defined causal agents, several drugs have been implicated including furosemide, spironolactone, sulphasalazine, penicillins, beta-blockers, penicillamine, antipsychotics, enoxoparin, DPP-4 inhibitors (Gliptins), and checkpoint inhibitors. Such patients tend to present at a younger average age. Prompt cessation of the drug results in rapid improvement. […] Bullous pemphigoid can occur in association with lichen planus.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

• #60 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. […] We don’t know what causes bullous pemphigoid. But we do know that it starts when your body’s immune system (your body’s defense against infection) makes antibodies (proteins) that attack healthy cells. This is called an autoimmune response. […] Certain things have been linked to bullous pemphigoid, but we don’t think they cause it. They include diseases, such as ulcerative colitis and multiple sclerosis, medications like furosemide and non-steroidal anti-inflammatory drugs (NSAIDs), and ultraviolet radiation and radiotherapy.

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

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

  Bullous pemphigoid occurs more often in patients age 60 but can occur in children. IgG autoantibodies bind to certain hemidesmosomal antigens (BPAg1 [BP230], BPAg2 [BP180]), resulting in the activation of complement to form a subepidermal blister. […] No cause of bullous pemphigoid has been proved; however, the following triggers have been suggested: Drugs (including furosemide, spironolactone, omeprazole, PD-1 and PD-L1 monoclonal antibodies [eg, durvalumab, nivolumab, pembrolizumab], sulfasalazine, penicillin, penicillamine, etanercept, antipsychotics, and dipeptidyl peptidase-4 inhibitors) […] Physical triggers (including trauma, radiation therapy for breast cancer, UV radiation, and anthralin) […] Skin disorders (including psoriasis, lichen planus, and some infections) […] Disorders (diabetes mellitus, rheumatoid arthritis, ulcerative colitis, and multiple sclerosis)

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

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

  Genetic and environmental factors may play a role. […] Triggers may induce an autoimmune reaction by mimicking molecular sequences in the epidermal basement membrane (molecular mimicry, as with drugs and possibly infections), by exposing or altering normally tolerated host antigens (as with physical triggers and certain disorders), or by other mechanisms. […] Certain central nervous system (CNS) and psychiatric disorders may precede bullous pemphigoid, especially multiple sclerosis and schizophrenia, but also dementias, intracranial bleeds, stroke, delusional and personality disorders, and Parkinson disease. To a lesser degree, these disorders may be preceded by bullous pemphigoid. Hypothesized shared causes include a cross-reactive immune response between neural and cutaneous antigens (BPAg1 is expressed in the CNS), as well as triggering by certain drugs used to treat the CNS disorders (eg, phenothiazine antipsychotics, spironolactone); however, a mechanism of triggering by drugs is not understood.

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

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

  Dipeptidyl peptidase 4 (DPP-4) inhibitors have been associated with a risk of developing bullous pemphigoid. […] Environmental factors have been associated with bullous pemphigoid, including daily consumption of green or herbal tea, fish oil, calcium supplements, multivitamins, and glucosamine. Other triggers associated with bullous pemphigoid include use of lime-containing household cleaning products and high levels of mental stress.

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

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

  Dipeptidyl peptidase 4 (DPP-4) inhibitors have been associated with a risk of developing bullous pemphigoid. […] Environmental factors have been associated with bullous pemphigoid, including daily consumption of green or herbal tea, fish oil, calcium supplements, multivitamins, and glucosamine. Other triggers associated with bullous pemphigoid include use of lime-containing household cleaning products and high levels of mental stress.

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

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

  Dipeptidyl peptidase 4 (DPP-4) inhibitors have been associated with a risk of developing bullous pemphigoid. […] Environmental factors have been associated with bullous pemphigoid, including daily consumption of green or herbal tea, fish oil, calcium supplements, multivitamins, and glucosamine. Other triggers associated with bullous pemphigoid include use of lime-containing household cleaning products and high levels of mental stress.

• #66 Bullous Pemphigoid | IntechOpen

  https://www.intechopen.com/chapters/59076

  Bullous pemphigoid (BP) is a chronic, acquired, autoimmune bullous disease characterized by subepidermal bullae. […] Autoantibodies against hemidesmosomal proteins BP230 (BPAG1) and BP180 (BPAG2 or type XVII collagen) are blamed for the pathogenesis. […] Autoantibodies against hemidesmosomal proteins BP230 (BPAG1) and BP180 (BPAG2 or type XVII collagen) are blamed for the pathogenesis. […] It has been shown that furosemide, psoralen, ibuprofen, galantamine, hydrobromide, ACEi, spironolactone, penicillin, levofloxacin, metronidazole may lead to BP. […] Adalimumab and etanercept (TNF-alpha blockers) associated BP cases have also been reported. […] Another cause blamed in the etiology is vaccinations. BP may develop after 1 day-4 weeks of vaccination. […] In addition, trauma, radiotherapy and UVB exposure may cause bullous lesions by uncovering BP antigens.

• #67 Pathology Outlines – Bullous pemphigoid

  https://www.pathologyoutlines.com/topic/skinnontumorbullouspemphigoid.html

  Most common autoimmune blistering skin disorder […] Autoimmune disease associated with bullous pemphigoid antigen 180 (BP180, BPAG2) and bullous pemphigoid antigen 230 (BP230, BPAG1, BPAG1e) (Am J Clin Dermatol 2017;18:513) […] Associated with medication use: […] Pediatric cases associated with viral illness and immunization (BMC Pediatr 2017;17:60)

• #68 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  The term pemphigoids includes a group of autoimmune bullous diseases characterized by subepidermal blistering. Bullous pemphigoid (BP) is not only the most common disorder within the pemphigoid group, but also represents the most frequent autoimmune blistering disease in general. The onset and course of BP depend on a variable interaction between predisposing and inducing factors. HLA genes are the most significant genetic predisposition factor to autoimmunity mechanisms. Many studies show an association between HLA-DQ1*0301 and distinct clinical pemphigoid variants. Imbalance between autoreactive T helper (Th) and T regulatory cells, toll-like receptor activation, and Th17/IL-17 pathway are the three possible autoimmunity triggers underlying BP. […] The intervention of inducing factors in BP can be identified in no more than 15% of patients. Facilitating factors in genetically predisposed individuals are various (drug intake, physical agents, and viral infections). Drugs may act as triggers by either modifying the immune response or altering the antigenic properties of the epidermal basement membrane.

• #69 Bullous Pemphigoid: Trigger and Predisposing Factors

  https://www.mdpi.com/2218-273X/10/10/1432

  Bullous pemphigoid (BP) is the most frequent autoimmune subepidermal blistering disease provoked by autoantibodies directed against two hemidesmosomal proteins: BP180 and BP230. Its pathogenesis depends on the interaction between predisposing factors, such as human leukocyte antigen (HLA) genes, comorbidities, aging, and trigger factors. Several trigger factors, such as drugs, thermal or electrical burns, surgical procedures, trauma, ultraviolet irradiation, radiotherapy, chemical preparations, transplants, and infections may induce or exacerbate BP disease. […] The etiopathogenesis of BP is largely unknown, but in several cases the occurrence or exacerbation of the disease has been reported in association with a specific “trigger factor”, such as drugs, physical factors, vaccines, infections, transplantations, and others. […] The aim of the present review is to highlight the trigger and predisposing factors underlying the onset of BP.

• #70 Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23806156/

  A contributing role in inducing BP has been suggested for infections, in particular human herpes virus (HHV) infections (cytomegalovirus, Epstein-Barr virus, and HHV-6), but also hepatitis B and C viruses, Helicobacter pylori, and Toxoplasma gondii. Unlike pemphigus, no dietary triggers have been suspected of being involved in the induction of BP. In all patients who have a diagnosis of BP, an environmental agent as a potential cause should always be considered, because the prompt discontinuation of it might result in rapid improvement or even cure of the disease.

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