Skin nontumor
Vesiculobullous and acantholytic reaction patterns
Epidermolysis bullosa acquisita


Topic Completed: 1 July 2011

Minor changes: 23 July 2020

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PubMed Search: Epidermolysis bullosa acquisita

Nat Pernick, M.D.
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Cite this page: Pernick N. Epidermolysis bullosa acquisita. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skinnontumoreba.html. Accessed August 11th, 2020.
Definition / general
  • Rare, noncongenital, 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
Microscopic (histologic) description
Immunofluorescence 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
Videos

Epidermolysis bullosa acquisita

Differential diagnosis
  • Bullous pemphigoid: mild dermal infiltrate including eosinophils; reactivity on epidermal side in NaCl split skin
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