Skin-nontumor / Clinical dermatology
Blistering disorders
Epidermolysis bullosa acquisita

Author: Nat Pernick, M.D. (see Authors page)

Revised: 14 June 2016, last major update July 2011

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Epidermolysis bullosa acquisita

Cite this page: Epidermolysis bullosa acquisita. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skinnontumoreba.html. Accessed December 9th, 2016.
Definition / General
  • Rare, non-congenital, autoimmune, chronic listering disease of skin and mucus membranes (eMedicine)
Etiology
  • Usually IgG autoantibodies against NC1 (noncollagenous domain of type VII collagen), major component of anchoring fibrils that connect basement membrane to dermal structures; also antibodies to central triple-helical (collagenous) domain of type VII collagen and IgA antibodies instead of IgG
Clinical Features
  • Occurs at any age, usually affects elderly
  • Blisters, scars and milia at trauma prone areas
  • Some patients have generalized inflammatory skin blister phenotype
  • Chronic disorder with partial remissions and exacerbations
  • Causes significant morbidity but death due to disease is rare
Treatment
  • Corticosteroids and immunosuppressants, but relatively resistant to treatment
Micro Description
Micro Images

Images hosted on other servers:

Direct immunofluorescence on perilesional skin: linear band of IgG along dermal-epidermal junction



Indirect immunofluorescence on salt-split normal human skin substrate using serum from affected patient: IgG autoantibodies on dermal side of basement membrane

Positive Stains
  • Direct immunofluorescence on perilesional skin shows linear band of IgG along dermal-epidermal junction
  • Indirect immunofluorescence on salt-split normal human skin substrate using serum from affected patient shows IgG autoantibodies on dermal side of basement membrane
Differential Diagnosis
  • Bullous pemphigoid: mild dermal infiltrate including eosinophils; reactivity on epidermal side in NaCl split skin