Skin-nontumor / Clinical Dermatology - Epidermolysis bullosa acquisita

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Skin-nontumor / Clinical Dermatology

Blistering disorders

Epidermolysis bullosa acquisita

Reviewer: Nat Pernick, M.D., PathologyOutlines.com, Inc. (see Reviewerspage)
Revised: 26 August 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Rare, non-congenital, autoimmune, chronic listering disease of skin and mucus membranes (eMedicine)

Etiology
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● 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
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● 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
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● Corticosteroids and immunosuppressants, but relatively resistant to treatment

Clinical images
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Various images

Micro description
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● Subepidermal blister with mixed inflammatory cell dermal infiltrate
● Often has bullous pemphigoid-like features (Acta Derm Venereol 2011;91:307)

Micro images
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Subepidermal blister with no/rare inflammatory cells

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
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● 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
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● Dr. Julia Lehman, Mayo Clinic, Dr. Wendy Levinbook

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

End of Skin-nontumor / Clinical Dermatology > Blistering disorders > Epidermolysis bullosa acquisita)

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