Immunbullous Disease - Schwarzenberger Flashcards
Epidemiology of Bullous pemphigoid?
Affects older people, 68-82; Women predominant
What is the pathophysiology of bullous pemphigoid?
Autoantibodies against the hemidesmosomes in the basal keratinocytes –> BP 180 and BP 230 proteins
Describe the lesions seen EARLY in bullous pemphigoid
very itchy, urticarial rash
Describe the blisters of bullous pemphigoid. Where are they located?
Tense (stay taut with pressure), normally symmetric in distribution. Might have erythema
Picture of bullous pemphigoid
Mucosal involvement in bullous pemphigoid?
20% of cases; eye involvement can cause blindness, oral lesions common
What is gestational pemphigoid? What risks are associatesd with it?
Bullous pemphigoid arising in a pregnant woman; can cause preterm labor or low weight babies
What will skin biopsy of bullous pemphigoid show when immunofluoresced
IgG and C3 deposition at the dermal-epidermal junction (LINEAR PATTERN)
Bullous Pemphigoid and antibodies in blood?
May or may not find antibodies in the blood
What is the treatment for bullous pemphigoid?
Long term systemic steroids and/or immunosuppresive Rx; requires aggressive tx
Epidemiology of Pemphigus vulgaris?
All ages (mean onset 50-60); male=female; incdience higher in Ashkenazi Jews and those of Mediterranean descent
Pathophysiology of Pemphigus Vulgaris
Autoantibodies against the desmoglein 1 and 3
What is the characteristic thing seen on histology of pemphigus vulgaris?
“Row of Tombostones”, intraepidermal split blisters just above the dermis
Who frequently diagnoses pemphigus vulgaris?
Dentists (LOL); commonly starts as mucosal erosions
Describe the blisters seen in pemphigus vulgaris. Where are they located?
Flaccid and easily broken; commonly on the head, chest, and back
Are there serum antibodies in pemphigus vulgaris?
Yes; their serum concentration (of desmoglein 1 & 3) can serve as marker for disease progression
What is Nikolksy sign?
When the top layers of skin slip away from the underlying layers upon light pressure
+ in Pemphigus Vulgaris (…and Stevens Johnson Syndrome/TEN)
- in Bullous Pemphigoid (tense blister, doesnt sheer laterally under pressure)
In Pemphigus vulgaris, where do you want to take the skin biopsy from?
The very outer edge of inflammation/erosion, adjacent to the normal skin
What specific drug has show great efficacy in pemphis vulgaris?
Rituximab
What is Dermatitis herpetiformis?
Autoimmune bullous disease linked to celiac’s and gluten hypersensitivity
Dermatitis Herpetiformis: What type are the autoantibodies and what do they react with?
IgA; against tissue transglutaminase and cross react with epidermal transglutaminase
- Pt develops IgA antibodies to tissue transglutaminae (t-TG in gut)
- t-TG cross reacts with epidermal transglutaminase (e-TG in skin)
Where is transglutaminase found in the skin?
Dermal capillaries and the basal cells of the epidermis
Epidemiology of dermatitis herpetiformis
Men>women; onsets between 30-40
Mucosal involvement in dermatitis herpetiformis?
NO; helps you distinguish between the other bullous diseases
Describe the lesions in dermatitis herpetiformis. Where are they located?
Intensely itchy rash, possibly vesicular; commonly presents on the elbows, buttocks, knees, scalp, and neck
Relation between dermatitis herpetiformis and gluten sensitive enteropathy
ALL those that have dermatitis herpetiformis also have gluten sensitive disease; not vice versa
Histology of dermatitis herpetiformis
Lots of neutrophils, eosinophils infiltrating the dermal papillae
Treatment of dermatitis herpetiformis
Strict adherence to gluten free diet; steroids are NOT helpful
Dapsone used to treat which of these 3 diseases? Mechanism of action?
Treat Dermatitis herpetiformis. Prevents neutrophil infiltration into dermal papillae
What disease associations does dermatitis herpetiformis have?
Non-hodgkin’s lymphoma (most be worked up); other autoimmune disorders, Thyroid disease, DM, connective tissue disease
