Podcast
Questions and Answers
Which of the following is the most common subepidermal autoimmune blistering disease?
Which of the following is the most common subepidermal autoimmune blistering disease?
Which antibody is considered to be pathogenic in bullous pemphigoid?
Which antibody is considered to be pathogenic in bullous pemphigoid?
What is the typical age demographic affected by bullous pemphigoid?
What is the typical age demographic affected by bullous pemphigoid?
Which of the following is a common clinical presentation of non-bullous bullous pemphigoid?
Which of the following is a common clinical presentation of non-bullous bullous pemphigoid?
Signup and view all the answers
What is a typical characteristic of the bullae in the classic presentation of bullous pemphigoid?
What is a typical characteristic of the bullae in the classic presentation of bullous pemphigoid?
Signup and view all the answers
What is a common symptom associated with bullous pemphigoid?
What is a common symptom associated with bullous pemphigoid?
Signup and view all the answers
When does pemphigoid gestationis typically begin during pregnancy?
When does pemphigoid gestationis typically begin during pregnancy?
Signup and view all the answers
Which of the following is considered a standard initial therapy for moderate to severe Pemphigus vulgaris?
Which of the following is considered a standard initial therapy for moderate to severe Pemphigus vulgaris?
Signup and view all the answers
Besides pregnancy, what other factors can cause a flare-up of pemphigoid gestationis?
Besides pregnancy, what other factors can cause a flare-up of pemphigoid gestationis?
Signup and view all the answers
What is the primary role of oral immunosuppressants in the treatment of Pemphigus vulgaris?
What is the primary role of oral immunosuppressants in the treatment of Pemphigus vulgaris?
Signup and view all the answers
What was the life expectancy of a patient with Pemphigus Vulgaris before prednisone treatment was available?
What was the life expectancy of a patient with Pemphigus Vulgaris before prednisone treatment was available?
Signup and view all the answers
Which of these is NOT classified as a subtype of pemphigus?
Which of these is NOT classified as a subtype of pemphigus?
Signup and view all the answers
What is the primary enzyme associated with Dermatitis Herpetiformis (DH)?
What is the primary enzyme associated with Dermatitis Herpetiformis (DH)?
Signup and view all the answers
What percentage of patients treated with Rituximab achieve remission off steroids after 2 years of treatment?
What percentage of patients treated with Rituximab achieve remission off steroids after 2 years of treatment?
Signup and view all the answers
Which of the following is the most common presenting symptom in patients newly diagnosed with DH?
Which of the following is the most common presenting symptom in patients newly diagnosed with DH?
Signup and view all the answers
What is the typical distribution pattern of skin lesions in Dermatitis Herpetiformis (DH)?
What is the typical distribution pattern of skin lesions in Dermatitis Herpetiformis (DH)?
Signup and view all the answers
Which of the following is considered a very long-term side effect of Oral steroid use?
Which of the following is considered a very long-term side effect of Oral steroid use?
Signup and view all the answers
What is the most direct treatment for patients with pemphigus that are only showing minimal symptoms?
What is the most direct treatment for patients with pemphigus that are only showing minimal symptoms?
Signup and view all the answers
Which blood test is most specific for diagnosing DH?
Which blood test is most specific for diagnosing DH?
Signup and view all the answers
Which is most associated with Pemphigus Vulgaris?
Which is most associated with Pemphigus Vulgaris?
Signup and view all the answers
What is the primary treatment for the underlying cause of Dermatitis Herpetiformis (DH)?
What is the primary treatment for the underlying cause of Dermatitis Herpetiformis (DH)?
Signup and view all the answers
Which medication provides rapid relief from pruritus associated with Dermatitis Herpetiformis (DH)?
Which medication provides rapid relief from pruritus associated with Dermatitis Herpetiformis (DH)?
Signup and view all the answers
What is a crucial prerequisite to assess before beginning treatment with dapsone for Dermatitis Herpetiformis?
What is a crucial prerequisite to assess before beginning treatment with dapsone for Dermatitis Herpetiformis?
Signup and view all the answers
What best describes the nature of intestinal symptoms in Dermatitis Herpetiformis (DH)?
What best describes the nature of intestinal symptoms in Dermatitis Herpetiformis (DH)?
Signup and view all the answers
Which of the following is the most common variant of pemphigus?
Which of the following is the most common variant of pemphigus?
Signup and view all the answers
Pemphigus vulgaris is characterized by autoantibodies targeting which of the following?
Pemphigus vulgaris is characterized by autoantibodies targeting which of the following?
Signup and view all the answers
Which of the following is a typical clinical presentation of pemphigus vulgaris?
Which of the following is a typical clinical presentation of pemphigus vulgaris?
Signup and view all the answers
Which of the following is the most appropriate medium to send a biopsy for direct immunofluorescence in the diagnosis of pemphigus?
Which of the following is the most appropriate medium to send a biopsy for direct immunofluorescence in the diagnosis of pemphigus?
Signup and view all the answers
Which of the following serological tests is highly sensitive and specific for circulating desmoglein 3 antibodies, often used in the diagnosis of pemphigus vulgaris?
Which of the following serological tests is highly sensitive and specific for circulating desmoglein 3 antibodies, often used in the diagnosis of pemphigus vulgaris?
Signup and view all the answers
Which of the following is a medication that is more likely to induce pemphigus foliaceus?
Which of the following is a medication that is more likely to induce pemphigus foliaceus?
Signup and view all the answers
Which of the following best describes the demographic at highest risk of developing pemphigus vulgaris?
Which of the following best describes the demographic at highest risk of developing pemphigus vulgaris?
Signup and view all the answers
Which of the following statements regarding skin findings in pemphigus vulgaris is correct?
Which of the following statements regarding skin findings in pemphigus vulgaris is correct?
Signup and view all the answers
A mother with bullous pemphigoid may have an increased risk for which of the following conditions?
A mother with bullous pemphigoid may have an increased risk for which of the following conditions?
Signup and view all the answers
Which of the following is a common medication associated with bullous pemphigoid?
Which of the following is a common medication associated with bullous pemphigoid?
Signup and view all the answers
What is the preferred medium to transport a skin biopsy for direct immunofluorescence (DIF) in the diagnosis of bullous pemphigoid?
What is the preferred medium to transport a skin biopsy for direct immunofluorescence (DIF) in the diagnosis of bullous pemphigoid?
Signup and view all the answers
Which of the following statements about ELISA testing for bullous pemphigoid is correct?
Which of the following statements about ELISA testing for bullous pemphigoid is correct?
Signup and view all the answers
For mild and localized bullous pemphigoid, what is generally considered the first-line treatment?
For mild and localized bullous pemphigoid, what is generally considered the first-line treatment?
Signup and view all the answers
What is a common risk factor for relapse of bullous pemphigoid after cessation of treatment?
What is a common risk factor for relapse of bullous pemphigoid after cessation of treatment?
Signup and view all the answers
Which of the following best describes the typical course of bullous pemphigoid?
Which of the following best describes the typical course of bullous pemphigoid?
Signup and view all the answers
Dermatitis herpetiformis is associated with which underlying condition?
Dermatitis herpetiformis is associated with which underlying condition?
Signup and view all the answers
Study Notes
Vesiculobullous Disorders
- Vesiculobullous disorders are a group of skin conditions characterized by blisters.
- Adherens junctions and desmosomes are cell-to-cell junctions, important for skin integrity. Desmosomes use keratin, cadherin, plakoglobin, desmoglein, desmocollin, plakophilin, and desmoplakin.
- Pemphigus is an autoimmune blistering disease. Different types exist, including vulgaris, vegetans, foliaceus, erythematosus, fogo selvagem, drug-induced, paraneoplastic, and IgA pemphigus.
- Pemphigus vulgaris is the most common type. It is an autoimmune blistering disease with antibodies directed toward desmoglein 3. Older adults (50-60s) are affected, with a 10-fold increased risk in individuals of Jewish ancestry. Symptoms include painful oral and/or other mucosal sites, potentially life-threatening.
- Pemphigus vulgaris diagnosis involves biopsy (H&E and DIF), and blood tests (IIF and ELISA). ELISA is highly sensitive and specific for desmoglein 3 antibodies.
Classification of Pemphigus
- Pemphigus vulgaris
- Pemphigus vegetans
- Pemphigus foliaceus
- Pemphigus erythematosus
- Fogo selvagem
- Drug-induced pemphigus
- Paraneoplastic pemphigus
- IgA pemphigus
Pemphigus Vulgaris - Clinical
- Characterized by painful oral and other mucosal erosions, sometimes with flaccid skin blisters.
- Mucosal erosions are irregular, and painful. Oral, esophageal, conjunctival, vaginal, penile, and anal mucosa can be involved.
- Skin blisters are easily ruptured and heal slowly, or not at all.
- Nikolsky and Asboe-Hansen signs are often present.
- Secondary infections (e.g., HSV, staph, strep) are common.
- Non-scarring lesions are a hallmark.
Pemphigus Vulgaris - Diagnosis
- Diagnosis requires biopsy (H&E and DIF).
- Indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA) on blood are used. IIF uses monkey esophagus, ELISA tests for desmoglein 3 antibodies. Antibody levels can predict disease activity.
Pemphigus Vulgaris - Treatment
- Prednisone is a cornerstone treatment.
- Immunosuppressants (e.g., mycophenolate, azathioprine, cyclophosphamide) can also be used.
- Rituximab is sometimes used, particularly in severe cases.
- Initial treatment varies from mild to moderate or severe pemphigus. Often includes oral steroids and, potentially, rituximab. Disease control can lead to tapering doses. Relapses may require increasing doses or additional treatment.
- Prognosis is influenced by steroid use before treatment. Without treatment, life expectancy was limited.
Subpidermal Bullous Diseases
- Includes bullous pemphigoid, pemphigoid gestationis, cicatricial pemphigoid, linear IgA disease, chronic bullous disease of childhood, dermatitis herpetiformis, bullous lupus, and epidermolysis bullosa acquisita.
- Bullous pemphigoid is the most common subepidermal autoimmune blistering disorder. It impacts older adults (age > 60).
- Characteristics include non-bullous or bullous presentation. Pruritus, excoriations, eczematous, papular or urticarial form are symptoms. Tense blisters on normal or erythematous skin. Oral involvement is present in about 10–30% of patients. Associated with peripheral eosinophilia.
- Diagnosis involves blood tests (ELISA, IIF) and biopsy (H&E and DIF). Topical or oral steroids, immunosuppressants, IVIg, or rituximab are treatment options.
Pemphigoid Gestationis
- Also known as "Herpes Gestationis."
- Starts during the second trimester of pregnancy. Often flares at delivery.
- Recurs during future pregnancies and periods of menstruation.
- Symptoms include pruritic, urticarial plaques and tense bullae, starting around the umbilicus and spreading.
- Risk to the baby includes prematurity or small size at birth.
- Maternal complications include an increased risk of Graves disease. There is a 10% risk to the infant of skin disease, but it usually resolves spontaneously.
Dermatitis Herpetiformis
- Cutaneous manifestation of celiac disease.
- Associated with northern European heritage and a mean age of around 40 years.
- Strong association with HLA-DQ2 (and celiac disease) and thyroid disorders (particularly Hashimoto thyroiditis)
- Characterized by symmetric grouped or herpetiform papulovesicles on an inflamed base, along with urticarial plaques and papules/vesicles.
- Areas affected include elbows, knees, extensor forearms, back, and buttocks. Severe pruritus (itching) is common. Lesions often rupture or erode, leaving crusted areas.
- Diagnosis requires biopsy (H&E and DIF) and blood tests (total serum IgA, IgA anti-endomysial, and anti-tissue transglutaminase antibodies).
- Treatment focuses on a gluten-free diet, supplemented by dapsone, a medication for quick relief of pruritus. No effect on intestinal disease, but lesions return within 24-48 hours of stopping if the patient continues to eat gluten.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Related Documents
Description
Explore vesiculobullous disorders, focusing on pemphigus, an autoimmune blistering disease. Learn about the role of adherens junctions, desmosomes, and diagnostic methods for pemphigus vulgaris. Understand the impact of this condition on older adults and populations with specific risk factors.
