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Epidermolysis Bullosa Acquisita (EBA)

A rare chronic autoimmune subepidermal blistering disease defined by autoantibodies against type VII collagen of anchoring fibrils; characterized by trauma-induced blisters and scarring on extensor surfaces, with milia formation and dystrophic scarring resembling hereditary dystrophic epidermolysis bullosa.

Written by: Saygı Hospital Health Guide Editorial Board
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This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is Epidermolysis Bullosa Acquisita (EBA)?

Epidermolysis bullosa acquisita (EBA) is a rare chronic autoimmune subepidermal blistering disease characterized by IgG autoantibodies (primarily IgG1 and IgG4 subclasses) directed against the non-collagenous-1 (NC1) domain of type VII collagen, which forms the anchoring fibrils that connect the basement membrane to the underlying dermis. Disruption of this anchorage causes blister formation at the level of the lamina densa, below the basement membrane.

Clinical phenotypes: (1) Classic mechanobullous (50–60 percent) — trauma-induced tense bullae and erosions on extensor surfaces (elbows, knees, knuckles, dorsum of hands and feet) with healing by atrophic scarring and milia, dystrophic or absent nails, scarring alopecia (closely resembles hereditary dystrophic EB but adult-onset); (2) Inflammatory BP-like (30–40 percent) — generalized pruritic vesiculobullous eruption, less scarring; (3) Mucous membrane pemphigoid-like (10–20 percent) — predominant oral, ocular, esophageal, laryngeal, anogenital involvement with cicatrizing complications; (4) Brunsting-Perry-like — head and neck lesions with scarring; (5) IgA EBA — purely IgA antibodies, milder course.

Diagnosis: lesional biopsy shows subepidermal blister with variable inflammatory infiltrate; perilesional direct immunofluorescence shows linear IgG (and often C3) at the BMZ; salt-split skin indirect IF localizes to dermal side (helps distinguish from BP which localizes to epidermal side); enzyme-linked immunosorbent assay (ELISA) for type VII collagen NC1 domain antibodies confirms diagnosis; transmission electron microscopy demonstrates blister cleavage below the lamina densa with absent or reduced anchoring fibrils.

Symptoms

Tense vesicles and bullae on trauma-prone areas (elbows, knees, knuckles, hands, feet)
Erosions and ulcers in trauma sites
Milia formation in healing lesions (white pinhead inclusion cysts)
Atrophic, dystrophic, or hypertrophic scarring
Nail dystrophy or loss
Scarring alopecia
Mucosal involvement: oral erosions, ocular conjunctivitis with symblepharon, esophageal strictures, laryngeal stenosis, anogenital scarring (in MMP-like variant)
Pruritus (especially in inflammatory BP-like form)

Risk Factors

Adult onset (mean age 40–50 years; can occur in children — childhood EBA)
African and African American descent (higher prevalence)
HLA-DR2 (HLA-DRB1*15:03) — strongest genetic association
Inflammatory bowel disease (Crohn disease in 30 percent — strong association, screen all EBA patients)
Autoimmune comorbidities (SLE, rheumatoid arthritis, autoimmune thyroid disease)
Hematologic malignancies (rare paraneoplastic association)
Diabetes mellitus, amyloidosis, multiple endocrine deficiency

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Recurrent blistering or erosions on trauma-prone areas (elbows, knees, hands, feet) in adults
  • Healing wounds leaving scars and white milia cysts
  • Nail loss or scarring alopecia developing without other cause
  • Mucosal blistering or erosions (oral, ocular, esophageal symptoms)
  • Known EBA with new mucosal symptoms or progression
  • Symptoms suggestive of inflammatory bowel disease (chronic diarrhea, weight loss) — Crohn screening indicated
  • Vision changes with conjunctival scarring (urgent ophthalmology)

Treatment Methods

01
Diagnostic: lesional and perilesional skin biopsy — H&E, direct immunofluorescence (linear IgG and C3 at BMZ), salt-split skin indirect IF (dermal side localization), serum ELISA for type VII collagen NC1 antibodies, transmission electron microscopy if available
02
Workup for associated diseases: gastrointestinal evaluation for inflammatory bowel disease (especially Crohn — colonoscopy if symptoms), autoimmune panel (ANA, dsDNA, RF, anti-CCP, thyroid function), CBC, BMP, screening for hematologic malignancy if clinically indicated
03
Management of mechanobullous form: trauma avoidance and protective dressings, wound care, dapsone 25–150 mg daily (G6PD pre-screening), colchicine 0.6 mg BID-TID (often first-line monotherapy or with dapsone)
04
Inflammatory BP-like or MMP-like forms: systemic corticosteroids (prednisone 0.5–1.5 mg/kg/day) plus steroid-sparing immunosuppressant
05
Steroid-sparing options: mycophenolate mofetil 1–1.5 g BID, azathioprine 1–2.5 mg/kg/day, cyclosporine, methotrexate (less commonly)
06
Refractory or severe disease: rituximab (1 g x 2 doses 2 weeks apart, B-cell depletion, may need re-treatment), IVIG 2 g/kg over 2–5 days monthly, plasmapheresis, immunoadsorption, cyclophosphamide (last resort)
07
Wound care: non-adherent dressings, topical antiseptics, infection prevention, moisture-retentive dressings; topical corticosteroids and tacrolimus for limited disease
08
Mucosal management: ophthalmology for ocular involvement (artificial tears, topical cyclosporine, surgery if scarring), gastroenterology for esophageal strictures (dilation), urology / gynecology for anogenital lesions
09
Long-term care: prognosis variable, often chronic with relapses; functional disability from scarring; multidisciplinary team essential (dermatology, ophthalmology, GI, ENT, oral medicine, plastic surgery, physical therapy, psychological support)

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.