epidermolysis bullosa aquisita
Jump to navigation
Jump to search
Introduction
Subepidermal bullous disease resembling bullous pemphigoid.
Etiology
- associated with:
Epidemiology
rare
Pathology
- subepidermal bulla with or without eosinophils or neutrophils
- IgG & C3 deposits along the epidermal basement membrane
- IgG deposits along the epidermal basement membrane may occur in the absence of C3 (contrast with bullous pemphigoid)
Clinical manifestations
- resembles bullous pemphigoid
- blisters or erosions induced by trauma
- lesions occur predomonantly on acral sites
Laboratory
- serum IgG anti-basement membrane antibodies detected by indirect immunofluorescence
- routine serum testing generally performed with monkey esophagus as substrate (25-50%)
- sensitivity increased by use of NaCl-split human skin as substrate
- antibodies localize to dermal side of substrate
- in bullous pemphigoid, antibodies localize epidermal & occasionally also dermal side of substrate
- biopsy:
- immunofluorescence testing of perilesional skin
- immunoelectron microscopy
Management
- treatment is generally unsatisfactory
- some patients may respond to systemic glucocorticoids either alone or in combination with immunosuppressive agents
- inflammatory lesions may respond to dapsone
- the chronic, non-inflammatory form is largely resistant to therapy