Vesiculobullous blistering Flashcards
what is the primary feature of immunobullous disorders
blisters
what is nikosky’s sign
the top layers of the skin slip away from each other when rubbed gently
what is nikosky’s sign postive
pemphigus vulgaris
pemphigus vulgaris
produces fragile fluid filled blisters that rupture to form shallow erosions
where is the split in pemphigus vulgaris
superficial and intraepidermal
where does pemphigus vulgaris typically effect
scalp, face, axillae and groin and oral area.
mucosal involvement eg eyes, genitals common
what is there a risk of when lesions rupture
infection 2y
pemphigus vulgaris pathpphysiology
- IgG antibodies made against desmoglein 3, immune complexes form and results in loss of intercellular connections (desmosomes). This results in loss of cohesion between keratinocytes – ACANTHOLYSIS
where is desmoglein 3 found
found in keratinocytes
Bullous Pemphigoid usually presents in
elderly , background of dermatitis or normal skin
Bullous Pemphigoid
dense blisters and erosions
where is the split in Bullous Pemphigoid
through DEJ
do Bullous Pemphigoid heal with/out scarring
without scarring
what is the prebullous period in Bullous Pemphigoid
can be prolonged - persistent pruritic urticated plaques or eczema precedes blisters
does mucous involvement occur in Bullous Pemphigoid
in about 20% cases
does acantholysis occur in Bullous Pemphigoid or pemphigus vulgaris
pemphigus vulgaris
Bullous Pemphigoid pathophysiology
Hemidesmosomes are found in the basement membrane zone, and act to keep the epidermis attached to the dermis. Circulating antibodies (IgG) attack the hemidesmosomes in the BM, causing the epidermis to float off from BM.
what are hemidesmosomes
The hemidesmosomes are found in the BM zone of epidmermis of skin and act to keep the epidermis attached to the dermis normally
what drugs can induce Bullous Pemphigoid
ACE inhibitors, penicillin and furosemide
Bullous Pemphigoid prognosis
chronic self limiting course . most patients achieve remission on treatment within 3-6 months
what is the mortality of pemphigus like if left untreated
high
investigations
skin biopsy with direct immunofluorescence and indirect immunofluorescence
Dermatitis Herpetiformis - genes
HLA DQ2 and DQ8
how is PV treated
PV: 60-100m daily oral prednisolone. IS agents are used as steroid sparing agents.
how is BP treated
- oral or systemic steroids
- antibiotics for 2y bacterial infection
- Mild disease can be treated with high dose oral prednisolone (30-60mg daily) and steroid-sparing agents
- tetracyclines (doxycycline) can be used as a steroid sparing agent
