Autoimmune Skin Disorder - Bullous Pemphigoid Flashcards
What is Bullous Pemphigoid?
Autoimmune condition causing sub-epidermal blistering of the skin.
What is Bullous Pemphigoid?
Autoimmune condition causing sub-epidermal blistering of the skin.
Clinical Features of Bullous Pemphigoid (3).
- Itchy Deep Tense Blisters Around Flexor Surfaces in Elderly.
- Blisters Heal Without Scarring.
- No Mucosal Involvement.
Investigation of Bullous Pemphigoid.
Skin Biopsy : Immunofluorescnce : IgG and C3 at Dermoepidermal Junction.
Management of Bullous Pemphigoid (3).
- Referral to Dermatology.
- Oral Corticosteroids - Mainstay.
- Other : Topical Corticosteroids, Immunosuppressants and Antibiotics.
Pathophysiology of Bullous Pempigoid.
Type II Hypersensitivity Reaction : Autoantibodies (against hemidesmosomal proteins BP180 and BP230) deposited on the Basement Membrane.
Clinical Features of Pemphigus Vulgaris.
- Fragile Superficial Blisters that Rupture Easily and Undergo De-Squamation.
- Nikolsky Sign is positive.
- Oral Mucosa is almost always affected.
Pathophysiology of Pemphigus Vulgaris.
Autoimmune condition where there is deposition of IgG autoantibodies (Desmoglein 3) within the epidermis - binding to desmosomes between keratinocytes and cleaving them allowing cells to separate and blisters to form.
Investigations of Pemphigus Vulgaris.
Diagnosis - Skin Biopsy and Immunofluorescence : Deposition of IgG Auto-Antibodies within Epidermis.
Management of Pemphigus Vulgaris.
Oral Corticosteroids to prevent death - not to cure disease.
What is Nikolsky’s Sign?
Spread of bull following application of horizontal tangential pressure to the skin.
Clinical Features of Bullous Pemphigoid (3).
- Itchy Deep Tense Blisters Around Flexor Surfaces in Elderly.
- Blisters Heal Without Scarring.
- No Mucosal Involvement.
Investigation of Bullous Pemphigoid.
Skin Biopsy : Immunofluorescnce : IgG and C3 at Dermoepidermal Junction.
Management of Bullous Pemphigoid (3).
- Referral to Dermatology.
- Oral Corticosteroids - Mainstay.
- Other : Topical Corticosteroids, Immunosuppressants and Antibiotics.
Pathophysiology of Bullous Pempigoid.
Type II Hypersensitivity Reaction : Autoantibodies (against hemidesmosomal proteins BP180 and BP230) deposited on the Basement Membrane.