Immune-Mediated Skin Disease Flashcards
define autoimmune disease
specific humoral or cell mediated immune repsonse against autoantigens -> disease
define immune-mediated disease
mediated by the immune system - innappropriate inflammation or immune attack where specific humoral or cellular response to SELF AG NOT demonstrated.
- excludes hypersesntivities (even those these are immune mediated technically)
are immune mediated diseases common?
no
what type of immune assault leads to vesicles and bullae? what other lesions may be seen associated with these?
basement membrane or basal keratinocytes targetted
(epidermis separates from underlying tissue)
- erosions and ulcers more commonly seen as vesicles fragile
egs. of bullous or vesicular disease
- bullous pemphigoid
- pemphigous vulgaris
(NB: pempigous folacious = pustular disease)
what is targetted in subepidermal vesicular autoimmune disease?
- basememnt membrane
- accumulation of inflammatory cells (neutrophils, eosinophils) may be seen in conjunction with 1* lesions
where are subepidermal vesicular autoimmune disease lesions commonly seen?
mucocutaneous junction, axilla, groin, mucous membranes
Is bullous pemphigoid a single disease?
NO! recent molecular studies -> multiple types of subepidermal vesicular dermatitides eg.
- BP (bullous pemphigoid) dogs, cats, pigs, horses
- MMP (mucous membrane pemphigoid) dogs, cats
- EBA (epidermal bullosa acquisita) dogs
what is targetted in pemphigus vulgaris?
desmosomes
Ddx subepidermal vesicular autoimmune diseases?
- other ulcerative autoimmune diseases
- drug reactions
- epitheliotropic lymphoma
Dx of subepidermal vesicular autoimmune disease?
- hx, cs
- biopsy (cell rich or cell poor subepidermal vesic. dermatitis)
What occours with intraepidermal vesicular dermatitis?
- autoAb to desmosomal adhesion molecules (desmogleins)
- separation of keratinocyutes (acantholysis)
- blister/pustule formation
eg. of intraepidermal vesicular dermatitis? What is seen in this condition?
- pemphigus vulgaris
- suprabasilar clefting (“tombstone” appearance on histo as basal keratinocytes still attached to basement membrane)
- oral cavity affected in 90% cases
- mucocutaneous junction, claws, ears, axilla, grouin
- 2* bacterial infection common
- fever and depression severe
Dx of pemphigus vulgaris?
- h, cs
- skin biopsy: cupra basilar split, tombstones, FEW inflam cells)
Tx and prognosis of pemphigus vulgaris?
- suppress immune response (See later questinos on general tx principles)
- prognosis poor, needs agressive tx, difficult to get on top of
Most common pemphigus disease? What type of disease is this?
- pemphigus folaceous
- intraepidermal pustular disease
Which animals are affected by pemphigus folaceous?
- dogs cats horses