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epidermolysis bullosa aquisita
Subepidermal bullous disease resembling bullous pemphigoid.
Etiology:
- associated with:
a) amyloidosis
b) multiple myeloma
Epidemiology: rare
Pathology:
1) subepidermal bulla with or without eosinophils or neutrophils
2) IgG & C3 deposits along the epidermal basement membrane
3) IgG deposits along the epidermal basement membrane may occur in the absence of C3 (contrast with bullous pemphigoid)
Clinical manifestations:
1) resembles bullous pemphigoid
2) blisters or erosions induced by trauma
3) lesions occur predomonantly on acral sites
Laboratory:
1) serum IgG anti-basement membrane antibodies detected by indirect immunofluorescence
a) routine serum testing generally performed with monkey esophagus as substrate (25-50%)
b) sensitivity increased by use of NaCl-split human skin as substrate
1] antibodies localize to dermal side of substrate
2] in bullous pemphigoid, antibodies localize epidermal & occasionally also dermal side of substrate
2) biopsy:
a) immunofluorescence testing of perilesional skin
b) immunoelectron microscopy
Management:
1) treatment is generally unsatisfactory
2) some patients may respond to systemic glucocorticoids either alone or in combination with immunosuppressive agents
3) inflammatory lesions may respond to dapsone
4) the chronic, non-inflammatory form is largely resistant to therapy
Related
bullous pemphigoid; parapemphigus
General
epidermolysis bullosa
vesiculobullous dermatitis
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 167, 172
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY,
1994, pg 288