Podcast
Questions and Answers
Which autoantibody is primarily responsible for the pathogenesis of bullous pemphigoid (BP)?
Which autoantibody is primarily responsible for the pathogenesis of bullous pemphigoid (BP)?
What distinguishes the mechanism of lesion formation in bullous pemphigoid compared to pemphigus vulgaris?
What distinguishes the mechanism of lesion formation in bullous pemphigoid compared to pemphigus vulgaris?
What is a common treatment option for autoimmune blistering diseases?
What is a common treatment option for autoimmune blistering diseases?
Which statement about pemphigus vulgaris (PV) and pemphigus foliaceus (PF) is accurate?
Which statement about pemphigus vulgaris (PV) and pemphigus foliaceus (PF) is accurate?
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Which of the following is true regarding the role of CD4+ T cells in the formation of anti-Dsg3 autoantibodies?
Which of the following is true regarding the role of CD4+ T cells in the formation of anti-Dsg3 autoantibodies?
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What distinguishes pemphigus vulgaris from pemphigus foliaceous in terms of clinical manifestation?
What distinguishes pemphigus vulgaris from pemphigus foliaceous in terms of clinical manifestation?
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Which option correctly describes the primary autoantigen targeted in pemphigus foliaceous?
Which option correctly describes the primary autoantigen targeted in pemphigus foliaceous?
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What is a common feature of bullous pemphigoid as compared to pemphigus?
What is a common feature of bullous pemphigoid as compared to pemphigus?
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What role do enzyme-linked immunosorbent assays (ELISAs) play in the diagnosis of autoimmune blistering diseases?
What role do enzyme-linked immunosorbent assays (ELISAs) play in the diagnosis of autoimmune blistering diseases?
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Which statement is accurate regarding the lesions in mucous membrane pemphigoid?
Which statement is accurate regarding the lesions in mucous membrane pemphigoid?
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Study Notes
Autoimmune Blistering Diseases
- Chronic, debilitating conditions causing skin and/or mucous membrane blisters or erosions.
- Some are fatal if untreated.
- Classification based on clinical features (lesion distribution, morphology).
- Pemphigus and pemphigoid are the most common types.
Pemphigus Subtypes
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Pemphigus vulgaris (PV):
- Characterized by flaccid bullae/erosions affecting skin and mucous membranes.
- Targets desmoglein 3 (Dsg3) as the primary autoantigen.
- Often presents with oral/esophageal lesions first, then skin.
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Pemphigus foliaceous (PF):
- Affects only the skin, presenting with fragile blisters or superficial erosions.
- Targets desmoglein 1 (Dsg1) as the primary autoantigen.
- Typically presents only in the skin.
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Other rare forms:
- IgA pemphigus (IgA autoantibodies against desmocollin 1, Dsg1 or Dsg3)
- Paraneoplastic pemphigus (autoantibodies against multiple desmosomal proteins plus T cells)
Bullous Pemphigoid (BP)
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Characterized by:
- Urticarial lesions progressing to tense bullae on skin.
- Less commonly, mucous membrane erosions.
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Primarily targets hemidesmosomal components.
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Primary autoantigens:
- Collagen XVII (COL17, BP180).
- Plakin family protein BP230 (BPAG2).
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COL17 (BP180) is the major target.
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Complement (C3) deposition crucial for blister formation in BP.
Diagnostic Methods
- Routine histology and immunofluorescence microscopy for diagnosis.
- Enzyme-linked immunosorbent assays (ELISAs) detect circulating autoantibodies.
Mechanisms and Treatment
- Autoimmune mechanisms: Loss of tolerance allowing the immune system to attack self-antigens.
- Treatment: Typically uses systemic glucocorticoids and/or immunosuppressive agents.
- Targeted therapies: Monoclonal antibody rituximab effective in targeting B cells in a targeted fashion reducing or eliminating relapses.
Disease Models
- Animal models (e.g., Dsg3 knockout [KO] mice) help study PV.
- Transfer of immune cells/molecules from these animal models helps elucidate the mechanisms of autoimmune blister formation.
Key antigens and their roles
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Desmogleins:
- Dsg3 in PV impacts various mucosal surfaces.
- Dsg1 in PF impacts merely epidermal locations.
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Collagen XVII (COL17):
- In BP, crucial in linking basal keratinocytes to the basement membrane.
- BP230/BPAG2: Intracellular protein with an uncertain pathogenic role in BP.
Unique features of various blistering conditions
- Pemphigoid gestationis: Identical clinical features and autoantibody profiles as BP only in pregnant women
- Mucous membrane pemphigoid: Circulating antibodies target various basement membrane proteins.
- Epidermolysis bullosa acquisita: Primarily targets type VII collagen (COL7).
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Description
Dive into the crucial aspects of autoimmune blistering diseases, focusing on classification, clinical features, and the common subtypes of pemphigus. Learn about pemphigus vulgaris and foliaceous, their pathophysiology, and presentation. Discover the implications of early diagnosis and treatment options available for these debilitating conditions.
