Blistering (Bullous) Disorders Flashcards
(26 cards)
What are Bullous diseases classified by?
Location: Intra-epidermal (Subcorneal, Suprabasal) and Sub-epidermal
What are the causes of Bullous diseases?
Immune-mediated (immunofluorescence) or related to infection or trauma
What is Pemphigus vulgaris?
A rare autoimmune disease affecting intercellular attachments within the epidermis and mucosal epithelium
What is the main feature of Pemphigus vulgaris?
Acantholysis (destruction of desmosomes and loosening of the epidermis)
What is the appearance of lesions in Pemphigus vulgaris?
Superficial blisters that easily rupture
What age group does Pemphigus vulgaris typically affect?
Middle age and older individuals
What are the different types of Pemphigus?
PV (Pemphigus vulgaris - suprabasal, IgG to Desmoglein 3), PF (Pemphigus foliaceus - subcorneal, IgG to Desmoglein 1), Paraneoplastic pemphigus
What areas are mainly affected in Pemphigus vulgaris?
Oral mucosa, scalp, face, axilla, groin, and trunk
What is the histological feature of Pemphigus vulgaris?
Suprabasal acantholytic blister with variable infiltration by lymphocytes, histiocytes, and eosinophils
What is seen in the immunofluorescence of Pemphigus vulgaris?
Immunofluorescence shows IgG & C3 with a fishnet-like pattern of intercellular IgG deposits localized to sites of acantholysis
What is Bullous Pemphigoid?
Autoimmune vesiculobullous disease
What is the pathogenesis of Bullous Pemphigoid?
Auto-antibodies against bullous pemphigoid antigen 2 (BPAG12) that connects the basal layer to the basement membrane (dermo-epidermal junction)
Who is generally affected by Bullous Pemphigoid?
Elderly people (>60yo)
What is the appearance of the lesions in Bullous Pemphigoid?
Tense bullae filled with clear fluid on an erythematous base
What is the size of lesions in Bullous Pemphigoid?
Lesions can be up to 5 cm and do not rupture easily
Where do lesions typically occur in Bullous Pemphigoid?
Inner aspects of the thigh, flexor surfaces of the forearm, axilla, groin, and lower abdomen
Is oral involvement seen in Bullous Pemphigoid?
Oral involvement is seen in 10-15% of cases, usually after cutaneous lesions
What type of bullae are seen in Bullous Pemphigoid?
Subepidermal non-acantholytic bullae
What are the early histological features of Bullous Pemphigoid?
Early lesions show perivascular infiltrate of lymphocytes, eosinophils, and neutrophils, dermal edema, and basal cell vacuolization
What does the vacuolated basal layer lead to in Bullous Pemphigoid?
The vacuolated basal layer eventually gives rise to a blister
What is seen in the immunofluorescence of Bullous Pemphigoid?
Immunofluorescence shows IgG & C3 with linear deposition of IgG to basement membrane proteins, forming tense bullae
What is Dermatitis Herpetiformis?
Grouped vesicles with intense pruritus
Which gender is more affected by Dermatitis Herpetiformis?
More common in males
What percentage of Dermatitis Herpetiformis cases are associated with celiac disease?
80% associated with celiac disease
