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Bullous Pemphigoid

Updated : January 30, 2024





Background

The majority of subepidermal immunobullous instances (80%) of immune-mediated subepidermal blisters disorders are bullous pemphigoid (BP). The majority of geriatric persons with bullous pemphigoid are aged between the ages of sixties and eighties. Bullous pemphigoid can appear clinically in a variety of ways, but the immunobullous skin condition is characterized by stiff bullae & acute widespread pruritus.

Bullous lesions may not be present in unusual instances, and this calls for a high level of clinical caution. Direct immunofluorescence will show the antibodies against the outer membrane zone, & eosin and hematoxylin staining on a biopsy will reveal a subepidermal cleft containing eosinophils.

Bullous pemphigoid can also be diagnosed with ELISA screening. However, typical remedies involve topical and systemic immunosuppressive drugs. Treatment varies depending on how severe the condition is. The outcome varies, and ongoing monitoring is frequently necessary.

Epidemiology

As was already established, those over 60 years old are more susceptible to BP. In the U.S., there are 6 to 13 new instances per million individuals each year, compared to 12 – 13 per million in Europe. It does not discriminate based on race and affects men and women equally.

Although rare, this condition may occasionally manifest in children. Patients with BP may carry particular HLA class II alleles, such as the allele DQB1*0301 in White patients, as well as the genotypes DRB1*1101, DRB1*04 & DQB1*0302 in Japanese patients.

Anatomy

Pathophysiology

The immunologic & inflammatory components make up the two main parts of the pathophysiology of BP. The immunologic components include antibodies against BP antigen 230 (BPAG1) & type XVII collagen or BPAG2 (BP antigen 180), two components of the basal keratinocyte hemidesmosomal proteins.

The adhesion components that support epithelial-stromal adherence depend heavily on these antigens. Mast cells and complement are activated when autoantibodies attach to their target antigen and cause inflammation. As a result, numerous inflammatory cells, including neutrophils & eosinophils, release proteases that harm the dermal-epidermal interface.

Etiology

Some cases of bullous pemphigoid are brought on by systemic drugs, while most cases are brought on by antibodies targeting proteins organized at the dermal-epidermal interface. Bullous pemphigoid brought on by medicine can develop 3 months after starting treatment, and younger people are more likely to experience it.

Diuretics like spironolactone and furosemide, amoxicillin, NSAIDs, PD-1/PD-L1 inhibitors, TNF-alpha blockers, and gliptins are among the medications that have been described in the literature to produce bullous pemphigoid-like outbreaks.

Genetics

 

 

Prognostic Factors

Bullous pemphigoid usually goes away on its own after a few months, although it can linger for up to five years. The above-mentioned treatments can alleviate the symptoms of itching and soreness.

Clinical History

Clinical History

Bullous pemphigoid can begin subacutely or acutely with a broad rash of tight blisters. Particularly in older individuals, significant pruritus is commonly present and could be the only symptom of the condition. Blisters may develop in some people after recurrent urticarial episodes. Numerous nonbullous, persistent, inflamed skin conditions, including lichen planus & psoriasis, have been linked to bullous pemphigoid.

Bullous pemphigoid has been linked to exposure to some medicines, x-ray therapy, and UV light. Ibuprofen, furosemide, and also other nonsteroidal anti-inflammatory drugs, penicillamine, captopril, & antibiotics are some of the medications linked to bullous pemphigoid. It has been noted that bullous pemphigoid might appear soon after immunization, especially in children.

Physical Examination

Physical examination

Bullous pemphigoid can manifest clinically in a variety of ways, including the following:

Bullous pemphigoid in its generalized form: The most typical manifestation is the generalized bullous type. Tense bullae can appear anywhere on the skin’s surface, but they seem to favor the skin’s flexural regions. When it does occur, oral & ophthalmic mucosal involvement is uncommon and has little clinical importance. Bullae can develop on both erythematous and normal-appearing body surfaces. Bullae typically heal without leaving scars or developing milia.

Bullous pemphigoid in the vesicular form: Vesicular forms are less frequent. It shows up as clusters of tiny, tense lesions, frequently on an erythematous or urticarial basis.

Bullous pemphigoid in the vegetative form: The vegetative form is extremely rare and manifests as vegetating plaques in intertriginous skin regions such as the neck, axillae, groin, & inframammary regions. Pemphigus vegetans and this bullous pemphigoid type are very similar.

Bullous pemphigoid generalized erythroderma form: This unusual manifestation may resemble psoriasis, generalized atopic dermatitis, or other erythrodermic exfoliative skin diseases. In individuals with this variation, vesicles and bullae may form.

Bullous pemphigoid in the urticarial form: Some bullous pemphigoid patients initially exhibit chronic urticarial infections before developing bullous outbreaks. Urticarial infections are the only signs of the disease in some patients. According to a report from China, erythema, papules, and plaques were the primary nonbullous clinical manifestations in roughly 30% of individuals with bullous pemphigoid.

Bullous pemphigoid in the nodular form: Pemphigoid nodularis, an uncommon type with clinical characteristics resembling prurigo nodularis, causes blisters to emerge on normal-appearing and nodular lesions.

Bullous pemphigoid in acaral form: The face, palms, and soles are the primary sites of the bullous lesions in childhood-onset bullous pemphigoid linked to vaccination.

Bullous pemphigoid in its young form: Blisters tend to appear often on the cheeks, soles of the feet, and palms of infants with bullous pemphigoid, seldom affecting the genital region. Of these baby, patients had widespread blisters in 60% of them.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential Diagnoses

Linear IgA Dermatosis

Erythema Multiforme

Epidermolysis Bullosa Acquisita

Epidermolysis Bullosa

Drug-Induced Bullous Disorders

Dermatitis Herpetiformis

Cicatricial (Mucous Membrane) Pemphigoid

Laboratory Studies

 

 

Imaging Studies

 

 

Procedures

 

 

Histologic Findings

 

 

Staging

 

 

Treatment Paradigm

Systemic corticosteroids are the mainstay of bullous pemphigoid treatment, however, the severity of the disease & comorbidities will ultimately determine how the condition is managed. Super-potent topical steroids like clobetasol may be utilized in older patients with a localized illness that affects less than 20 percent of their body’s surface area. Numerous cases have shown efficacy with topical steroids paired with nicotinamide with tetracycline, doxycycline, and minocycline.

Systemic prednisone at a dose of 0.5 – 1 mg/kg per day is advised for severe illness. The condition can be controlled with this dosage of systemic corticosteroids in about 2 weeks, and it can be gradually tapered off over a period of 6 to 9 months or more. The patient’s age, comorbidities, & side effects place restrictions on this treatment plan. Strong topical corticosteroids can reduce systemic adverse effects while controlling generalized BP.

When systemic corticosteroid therapies are contraindicated for a patient or when the condition is not controlled by steroids, immunosuppressive therapy is employed. Azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide, and chlorambucil are other substitute medications. Treatment-resistant patients can be treated with IVIG, omalizumab, or anti-CD20/rituximab if all previous treatments are unsuccessful.

Numerous ELISA-based investigations have demonstrated a correlation between serum IgG autoantibody levels to BP180 and illness severity. At the conclusion of treatment, a high BP180-NC16A ELISA value and positive direct immunofluorescence indicate a potential recurrence.

by Stage

 

 

by Modality

 

 

Chemotherapy

 

 

Radiation Therapy

 

 

Surgical Interventions

 

 

Hormone Therapy

 

 

Immunotherapy

 

 

Hyperthermia

 

 

Photodynamic Therapy

 

 

Stem Cell Transplant

 

 

Targeted Therapy

 

 

Palliative Care

 

 

Medication

 

 

Media Gallary

References

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

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

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Bullous Pemphigoid

Updated : January 30, 2024




The majority of subepidermal immunobullous instances (80%) of immune-mediated subepidermal blisters disorders are bullous pemphigoid (BP). The majority of geriatric persons with bullous pemphigoid are aged between the ages of sixties and eighties. Bullous pemphigoid can appear clinically in a variety of ways, but the immunobullous skin condition is characterized by stiff bullae & acute widespread pruritus.

Bullous lesions may not be present in unusual instances, and this calls for a high level of clinical caution. Direct immunofluorescence will show the antibodies against the outer membrane zone, & eosin and hematoxylin staining on a biopsy will reveal a subepidermal cleft containing eosinophils.

Bullous pemphigoid can also be diagnosed with ELISA screening. However, typical remedies involve topical and systemic immunosuppressive drugs. Treatment varies depending on how severe the condition is. The outcome varies, and ongoing monitoring is frequently necessary.

As was already established, those over 60 years old are more susceptible to BP. In the U.S., there are 6 to 13 new instances per million individuals each year, compared to 12 – 13 per million in Europe. It does not discriminate based on race and affects men and women equally.

Although rare, this condition may occasionally manifest in children. Patients with BP may carry particular HLA class II alleles, such as the allele DQB1*0301 in White patients, as well as the genotypes DRB1*1101, DRB1*04 & DQB1*0302 in Japanese patients.

The immunologic & inflammatory components make up the two main parts of the pathophysiology of BP. The immunologic components include antibodies against BP antigen 230 (BPAG1) & type XVII collagen or BPAG2 (BP antigen 180), two components of the basal keratinocyte hemidesmosomal proteins.

The adhesion components that support epithelial-stromal adherence depend heavily on these antigens. Mast cells and complement are activated when autoantibodies attach to their target antigen and cause inflammation. As a result, numerous inflammatory cells, including neutrophils & eosinophils, release proteases that harm the dermal-epidermal interface.

Some cases of bullous pemphigoid are brought on by systemic drugs, while most cases are brought on by antibodies targeting proteins organized at the dermal-epidermal interface. Bullous pemphigoid brought on by medicine can develop 3 months after starting treatment, and younger people are more likely to experience it.

Diuretics like spironolactone and furosemide, amoxicillin, NSAIDs, PD-1/PD-L1 inhibitors, TNF-alpha blockers, and gliptins are among the medications that have been described in the literature to produce bullous pemphigoid-like outbreaks.

 

 

Bullous pemphigoid usually goes away on its own after a few months, although it can linger for up to five years. The above-mentioned treatments can alleviate the symptoms of itching and soreness.

Clinical History

Bullous pemphigoid can begin subacutely or acutely with a broad rash of tight blisters. Particularly in older individuals, significant pruritus is commonly present and could be the only symptom of the condition. Blisters may develop in some people after recurrent urticarial episodes. Numerous nonbullous, persistent, inflamed skin conditions, including lichen planus & psoriasis, have been linked to bullous pemphigoid.

Bullous pemphigoid has been linked to exposure to some medicines, x-ray therapy, and UV light. Ibuprofen, furosemide, and also other nonsteroidal anti-inflammatory drugs, penicillamine, captopril, & antibiotics are some of the medications linked to bullous pemphigoid. It has been noted that bullous pemphigoid might appear soon after immunization, especially in children.

Physical examination

Bullous pemphigoid can manifest clinically in a variety of ways, including the following:

Bullous pemphigoid in its generalized form: The most typical manifestation is the generalized bullous type. Tense bullae can appear anywhere on the skin’s surface, but they seem to favor the skin’s flexural regions. When it does occur, oral & ophthalmic mucosal involvement is uncommon and has little clinical importance. Bullae can develop on both erythematous and normal-appearing body surfaces. Bullae typically heal without leaving scars or developing milia.

Bullous pemphigoid in the vesicular form: Vesicular forms are less frequent. It shows up as clusters of tiny, tense lesions, frequently on an erythematous or urticarial basis.

Bullous pemphigoid in the vegetative form: The vegetative form is extremely rare and manifests as vegetating plaques in intertriginous skin regions such as the neck, axillae, groin, & inframammary regions. Pemphigus vegetans and this bullous pemphigoid type are very similar.

Bullous pemphigoid generalized erythroderma form: This unusual manifestation may resemble psoriasis, generalized atopic dermatitis, or other erythrodermic exfoliative skin diseases. In individuals with this variation, vesicles and bullae may form.

Bullous pemphigoid in the urticarial form: Some bullous pemphigoid patients initially exhibit chronic urticarial infections before developing bullous outbreaks. Urticarial infections are the only signs of the disease in some patients. According to a report from China, erythema, papules, and plaques were the primary nonbullous clinical manifestations in roughly 30% of individuals with bullous pemphigoid.

Bullous pemphigoid in the nodular form: Pemphigoid nodularis, an uncommon type with clinical characteristics resembling prurigo nodularis, causes blisters to emerge on normal-appearing and nodular lesions.

Bullous pemphigoid in acaral form: The face, palms, and soles are the primary sites of the bullous lesions in childhood-onset bullous pemphigoid linked to vaccination.

Bullous pemphigoid in its young form: Blisters tend to appear often on the cheeks, soles of the feet, and palms of infants with bullous pemphigoid, seldom affecting the genital region. Of these baby, patients had widespread blisters in 60% of them.

Differential Diagnoses

Linear IgA Dermatosis

Erythema Multiforme

Epidermolysis Bullosa Acquisita

Epidermolysis Bullosa

Drug-Induced Bullous Disorders

Dermatitis Herpetiformis

Cicatricial (Mucous Membrane) Pemphigoid

 

 

 

 

 

 

 

 

 

 

Systemic corticosteroids are the mainstay of bullous pemphigoid treatment, however, the severity of the disease & comorbidities will ultimately determine how the condition is managed. Super-potent topical steroids like clobetasol may be utilized in older patients with a localized illness that affects less than 20 percent of their body’s surface area. Numerous cases have shown efficacy with topical steroids paired with nicotinamide with tetracycline, doxycycline, and minocycline.

Systemic prednisone at a dose of 0.5 – 1 mg/kg per day is advised for severe illness. The condition can be controlled with this dosage of systemic corticosteroids in about 2 weeks, and it can be gradually tapered off over a period of 6 to 9 months or more. The patient’s age, comorbidities, & side effects place restrictions on this treatment plan. Strong topical corticosteroids can reduce systemic adverse effects while controlling generalized BP.

When systemic corticosteroid therapies are contraindicated for a patient or when the condition is not controlled by steroids, immunosuppressive therapy is employed. Azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide, and chlorambucil are other substitute medications. Treatment-resistant patients can be treated with IVIG, omalizumab, or anti-CD20/rituximab if all previous treatments are unsuccessful.

Numerous ELISA-based investigations have demonstrated a correlation between serum IgG autoantibody levels to BP180 and illness severity. At the conclusion of treatment, a high BP180-NC16A ELISA value and positive direct immunofluorescence indicate a potential recurrence.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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

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

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