Blistering Skin Conditions Flashcards
Clinical features of an intra-epidermal blister
Thin roof so burst easily
Name of an intra-epidermal blister
Pemphigus
Name of a sub-epidermal blister
Pemphigoid
Clinical feature of a sub-epidermal blister
Thick roof so more likely to stay intact
What is a bullous pemphigoid
Subepidermal blister caused by an autoimmune reaction
When does bullous pemohigoid usually present
> 60
Associations with bullous pemphigoid
Neurological disease: stroke, dementia, Parkinson’s
Internal malignancy
Autoimmune diseases
Name some drugs that can cause bullous pemphigoid
Diuretics
Analgesics
Antibiotics
Potassium iodide
Good
Captopril
Pathophysiology of bullous pemphigoid
IgG reacts with the major or minor antigen of the hemidesmosomes anchoring basal cells to the basement membrane
Compliment activation causes interruption of the DEJ and the formation of a subepidermal blister
What kind of hypersensitivity reaction is bullous pemphigoid
Type 2
Symptoms of bullous pemphigoid
Pruritus
Well demarcated erythematous plaques before the blisters
Large tense extremely itchy blisters
Nikolskys sign
Lateral pressure on the skin next to the blister causes it to extend
Will bullous pemphigoid be nikolsky sign positive
NO - negative
Histology of bullous pemphigoid
Subepidermal blisters with inflammatory infiltrates
Immunofluorescence on bullous pemphigoid
IgG and complement deposited around the basal membrane
Local management of bullous pemphigoid
Prednisolone
Systemic management of bullous pemphigoid
Prednisolone
+/- tetracycline antibiotics
+/- antihistamine
Why do we use antihistamines for bullous pemphigoid
For sedating and anti-pruritic properties
Why do we use tetracyclines for bullous pemphigoid
As steroid sparing agents
What do we do if we get no response to treatment for bullous pemphigoid
Immunosuppression with azathioprine or methotrexate
What is the most common subtype of pemphigus
Pemphigus vulgaris
When does Pemphigus vulgaris usually present
Middle age 40-60
What kind of hypersensitivity reaction is Pemphigus vulgaris
Type 2
Pathophysiology of Pemphigus vulgaris
IgG antibodies against desmosomal proteins lead to loss of keratinocyte adhesion in the skin and mucous membranes
What is acantholysis
Loss of intercellular adhesion sites
Symptoms of Pemphigus vulgaris
Fluid filled, painful, fragile blisters
Rupture to form shallow eruptions
Where do Pemphigus vulgaris usually present
Skin: scalp, face, axillae, groin, trunk
Mucosa: mouth, resp tract
Will Pemphigus vulgaris be nikolsky sign positive
YES
Immunofluorescence of Pemphigus vulgaris
Chicken wire deposition of IgG within the epidermis
Local disease management of Pemphigus vulgaris
Topical steroids + anaesthetics
Systemic management of Pemphigus vulgaris
Prednisolone + azathioprine
What is dermatitis herpetiformis associated with
Coeliac disease
When does dermatitis herpetiformis usually present
15-40 years
What gene is dermatitis herpetiformis associated with
HLA-DQ2 halotype
Pathophysiology of dermatitis herpetiformis
IgA transglutimase antibodies target glial in component of gluten but cross react with connective tissue matrix proteins
Immune complexes form in in dermal papillae
Subepidermal blisters
Clinical presentation of dermatitis herpetiformis
Intensely itchy symmetrical lesions on an erythematous and swollen base
Where can dermatitis herpetiformis present
Extensor aspect of elbows and forearms
Buttocks and scapulae
Extensor aspects of knees
Face and scalp
Blood results for a patient with dermatitis herpetiformis
Anti-TTG
Immunofluorescence of dermatitis herpetiformis
Granular deposits of IgA in dermal papillae
Histology of dermatitis herpetiformis
Subepidermal blisters with papillary dermal micro-abscesses
Management of dermatitis herpetiformis
Gluten free diet +/- dapsone
Topical steroids for symptoms
Complication of dermatitis herpetiformis
Rare but significantly increased risk of small bowel lymphoma