Epidermolysis bullosa acquisita Flashcards
Epidermolysis bullosa acquisita has an increased incidence in which populations?
- East asians and african americans
Epidermolysis bullosa acquisita is a/w what diseases (most to least common)
- Crohn’s/IBD
- mutiple myeloma
- SLE
- RA
- diabetes
- thyroiditis
What is pathogenesis of Epidermolysis bullosa acquisita?
- IgG autoantibodies against NC1 domain of type VII collagen (major component of anchoring fibrils of lambina densa/sublamina densa)
What are the two clinical patterns of Epidermolysis bullosa acquisita?
- Classic mechanobullous EBA
- Inflammatory (BP-like) EBA
How does classic mechanobullous Epidermolysis bullosa acquisita present clinically?
- non-inflammatory bullae and erosions on acral/trauma prone sites (elbows, knees)
- may result in mitten deformities of hands
How do the classic mechanobullous Epidermolysis bullosa acquisita lesions heal?
- with atrophic scars, milia and dyspigmentation
Histopathology of Epidermolysis bullosa acquisita?
cell-poor subepidermal blisters
How does inflammatory (BP-like) Epidermolysis bullosa acquisita present?
- indistinguisable from BP
- can also mimic Mucous membrane pemphigoid
What is the histopathology of Inflammatory (BP-like) Epidermolysis bullosa acquisita?
Indistinguishable from BP
How do you distinguish Inflammatory (BP-like) Epidermolysis bullosa acquisita from BP?
with DIF serration pattern
- can also use salt-split skin DIF, IIF, immunoblotting, or ELISA
What will DIF of Inflammatory (BP-like) Epidermolysis bullosa acquisita show as opposed to BP?
- EBA: broad linear band of IgG (>C3) with u-serrated pattern along BMZ
- BP: the opposite: C3> IgG with u-serrated pattern
Treatment for epidermolysis bullosa acquisita and response to tx?
- can try steroids, immunosuppressants, dapsone, IVIG
Refractory to tx!
