Week 13- Vesicullo Bullous Disorders Flashcards
What are signs and symptoms of vesicullobullous disorders?
- Vesicles or Bullae
- Intra-Epithelial or Sub-Epithelial Blistering
- Erythema, Blisters, Erosions, Ulcers
- Pain, Dysphagia, Foetor
What are 2 common vesicullobullous disorders?
Pemphigus vulgaris
Mucous membrane pemphigoid
What is a vesiculobullous disroder?
- Chronic Inflammatory Disorder
- Auto-Antibodies target Structural Proteins of Desmosomal & Hemi-Desmosomal Plaques in Skin & Mucosa
- Formation of vesicles or bullae
What is this?
Mucous Membrane Pemphigoid
What is the most common vesiculobullous disorder?
Mucous membrane pemphigoid
What is the pathophysiology of mucous membrane pemphigoid?
- IgG &/or IgA Auto-Antibodies target Hemi-Desmosomes in Epithelial Basement Membrane Zone
- Loss of Connection between Basal Epithelial Cells & Dermis
- Sub-Epithelial Blistering
What are characteristics of mucous membrane pemphigoid?
- Thick-walled bullous lesions burst & form ulcers
- Last Several Days before Ulceration
- Oral Lesions usually Heal without Scarring
- Non-Keratinized Mucosa
- May be Blood-Filled
- Desquamative Gingivitis
- Erythematous & Hyperaemic
- Extends beyond Marginal Gingiva
What age is mucous membrane pemphigoid common and how long does it last?
Last Several Days
55-65 Years of Age
What are histopathological features of mucous membrane pemphigoid diagnosis from incisional biopsy?
- Perilesional sample
- Subepithelial bullae
- No acantholytic cells
- Epithelium detaches from underlying lamina propria
What is the glowing effect in this sample?
Autoantibody attacking membrane basement cell junction
What are 2 types of immunofluorescence?
- Direct (biopsy sample): frozen, section, microscopy
- Indirect (pts serum): serum, substrate (salt-split skin), microscopy
What is tx for mucous membrane pemphigoid?
- Refer for specialist care
- Topical or systemic corticosteroids
- Immunosuppressant therapy
- Maintenance of OH
What is this?
Pemphigus vulgaris
What are characteristics of pemphigus vulgaris?
- Potentially lethal- infection, fluid & protein loss, dysphagia
- Oral lesions precede skin
- Rare
- Chronic
- Can be drug induced
What is pathophysiology of pemphigus vulgaris?
- IgG Auto-Antibody Binding targets Desmosomal Proteins interfering with Cell Adhesion
- Acantholysis
- Intra-Epithelial Blistering
What are clinical features of pemphigus vulgaris?
- Painful, Fragile, Fluid-Filled Blisters
- Soft / Hard Palate, Buccal Mucosa, Lips & Gingivae
- Burst within Few Hours
- Shallow Ulcers / Erosions
What is the histopathology for pemphigus vulgaris following incisional biopsy?
- Perilesional Sample
- Acantholysis
- Intra-Epithelial Bullae
- Leucocytic Infiltrate in Lamina Propria
What does immunofluorescence indicate for pemphigus vulgaris?
IgG, IgM & C3 bound to Intercellular areas of Epithelium
What are the 2 biopsy samples used for?
Histopatholgy
Direct IMF microscpy
What can be used to diagnose vesiculubullous disorders?
Clinical features
Biopsy samples
Serum samples
What are other vesiculobullous disorders?
- Paraneoplastic Pemphigus (Haematological Malignancies)
- Erythema Multiforme
- Epidermolysis Bullosa
- Angina Bullosa Haemorrhagica
How is pemphigus vulgaris treated?
- Refer for specialist care
- Systemic corticosteroids (prednisolone 40-60mg daily)
- Immunosuppressive therapy
- Topical corticosteroids
- Maintenance of OH
What are immunofluorescent signs of mucous membrane pemphigoid?
Linear IgG & C3 along Epithelial Basement Membrane Zone
What are signs of erythemia multiforme?
Oral/labial erythema, blistering and ulceration
