Podcast
Questions and Answers
What type of skin lesions are characterized by flaccid bullae that may develop into crusty erosions?
What type of skin lesions are characterized by flaccid bullae that may develop into crusty erosions?
- Pemphigus foliaceus (correct)
- Eczema
- Psoriasis
- Seborrheic keratosis
In which areas of the body are the lesions predominantly found?
In which areas of the body are the lesions predominantly found?
- Neck and arms
- Hands and feet
- Seborrheic areas like chest and face (correct)
- Lower back and abdomen
What is a common histopathological finding in pemphigus vulgaris?
What is a common histopathological finding in pemphigus vulgaris?
- Acantholysis at the granular layer
- Absence of inflammatory cells
- Intraepidermal suprabasal acantholysis (correct)
- Dyskeratotic keratinocytes
What is noted about the itchiness associated with the lesions in this condition?
What is noted about the itchiness associated with the lesions in this condition?
Which of the following best describes the transformation of lesions in pemphigus foliaceus?
Which of the following best describes the transformation of lesions in pemphigus foliaceus?
What kind of changes can be observed in the epidermis during histopathological examination?
What kind of changes can be observed in the epidermis during histopathological examination?
What involvement may occur in pemphigus that affects the nails?
What involvement may occur in pemphigus that affects the nails?
What is the recommended method for obtaining samples for histopathological analysis?
What is the recommended method for obtaining samples for histopathological analysis?
What is the primary goal of starting treatment in pemphigus patients?
What is the primary goal of starting treatment in pemphigus patients?
Which scoring system is used to measure the extent and severity of pemphigus lesions?
Which scoring system is used to measure the extent and severity of pemphigus lesions?
What factors should be evaluated to assess prognosis in pemphigus patients?
What factors should be evaluated to assess prognosis in pemphigus patients?
Which treatment is almost no longer used for drug-induced pemphigus?
Which treatment is almost no longer used for drug-induced pemphigus?
Which type of clinical involvement is critical to specify in pemphigus patients?
Which type of clinical involvement is critical to specify in pemphigus patients?
What is the recommended starting dosage of prednisone for tapering?
What is the recommended starting dosage of prednisone for tapering?
Which medications should be reviewed for potential induction of pemphigus?
Which medications should be reviewed for potential induction of pemphigus?
What is a contraindication for the use of live vaccines?
What is a contraindication for the use of live vaccines?
What measure is important for assessing the quality of life in pemphigus patients?
What measure is important for assessing the quality of life in pemphigus patients?
How can the tapering of prednisolone be performed?
How can the tapering of prednisolone be performed?
What is considered a significant risk factor when evaluating pemphigus patients?
What is considered a significant risk factor when evaluating pemphigus patients?
In which situation is the risk of skin relapse increased?
In which situation is the risk of skin relapse increased?
What does the tapering schedule of prednisone usually involve?
What does the tapering schedule of prednisone usually involve?
What should be done if a relapse occurs after stopping prednisone following rituximab treatment?
What should be done if a relapse occurs after stopping prednisone following rituximab treatment?
Which method is recommended for serological monitoring of disease activity?
Which method is recommended for serological monitoring of disease activity?
What should be done if ELISA for anti-Dsg1 and/or Dsg3 is not available?
What should be done if ELISA for anti-Dsg1 and/or Dsg3 is not available?
In non-rituximab treated patients, what is the recommended action during tapering of corticosteroids when a relapse occurs?
In non-rituximab treated patients, what is the recommended action during tapering of corticosteroids when a relapse occurs?
How do serum concentrations of IgG autoantibodies correlate with pemphigus?
How do serum concentrations of IgG autoantibodies correlate with pemphigus?
What is considered the first-line treatment for severe pemphigus when rituximab is ineffective?
What is considered the first-line treatment for severe pemphigus when rituximab is ineffective?
When might cyclophosphamide be considered in the treatment of pemphigus?
When might cyclophosphamide be considered in the treatment of pemphigus?
What is the recommended dosage of intravenous immunoglobulins (IVIG) for treatment cycles?
What is the recommended dosage of intravenous immunoglobulins (IVIG) for treatment cycles?
What precaution should patients on oral corticosteroids or immunosuppressive therapy take?
What precaution should patients on oral corticosteroids or immunosuppressive therapy take?
What type of corticosteroid injections may be used for isolated lesions of the oral mucosa in pemphigus patients?
What type of corticosteroid injections may be used for isolated lesions of the oral mucosa in pemphigus patients?
What topical treatment may be applied to oral erosions in some pemphigus patients?
What topical treatment may be applied to oral erosions in some pemphigus patients?
In which situation is rituximab applied as a second-line treatment?
In which situation is rituximab applied as a second-line treatment?
Which of the following is NOT a standard recommended vaccination for patients on immunosuppressive therapy?
Which of the following is NOT a standard recommended vaccination for patients on immunosuppressive therapy?
Study Notes
Pemphigus Treatment and Management
- Rituximab demonstrates higher complete remission rates compared to prednisone, with up to 58% achieving remission off therapy versus 34% with prednisone.
- Primary treatment options include steroids and steroid-sparing agents such as azathioprine and mycophenolate.
- Initial assessment of disease should include evaluating the extent of skin and mucosal involvement and any existing comorbidities.
- Prognosis is influenced by patient age and overall health status; complications can arise from immunosuppressive treatments.
- Lesion assessment using the Autoimmune Bullous Skin Intensity and Severity Score (ABSIS) or Pemphigus Disease Area Index (PDAI) is crucial for treatment planning.
- Dermatology-specific quality of life impacts should be measured using DLQI and other tools tailored to autoimmune blistering diseases.
Clinical Evaluation
- Record hematological, oncological, endocrine, and infectious histories to identify risk factors.
- Pemphigus typically features flaccid bullae that may progress to erosions, especially in seborrhoeic areas like the chest and scalp; it is usually not itchy.
- Nail involvement is possible, and lesions can emerge at mechanically stressed sites.
Histopathological Analysis
- Skin biopsies should be collected from perilesional areas for routine histopathology analysis to confirm diagnosis.
- Pemphigus vulgaris (PV) shows intraepidermal suprabasal acantholysis, while pemphigus foliaceus (PF) displays acantholysis at the granular layer.
Treatment Protocols
- In severe or refractory cases, adjunctive treatments such as intravenous immunoglobulins (IVIG) may be considered.
- High-potency topical corticosteroids (like clobetasol) can be applied directly to local oral erosions for symptomatic relief.
- Careful management of corticosteroid dosing is recommended, considering the risk of relapse correlated with anti-Dsg1 levels.
Monitoring and Follow-Up
- Regular serological monitoring for anti-Dsg1 and Dsg3 autoantibodies helps assess disease activity and guide treatment adjustments.
- If relapses occur during corticosteroid tapering, consider reintroducing rituximab treatments.
- Patients receiving immunosuppressive therapy should be vaccinated against common pathogens, avoiding live vaccines.
General Recommendations
- Adjust prednisone tapering schedules based on individual risk assessments and relapse history.
- Evaluate treatment response regularly to accommodate changes in disease activity and patient condition.
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Description
Explore the effectiveness of Rituximab compared to Prednisone in achieving complete remission in patients. This quiz focuses on identifying risk factors, severity factors, and potential co-morbidities based on clinical evaluations and historical data as reported in the JEADV 2020 study.
