EPIDERMOLYSIS BULLOSA ACQUISITA

By Gary M. White, MD

Epidermolysis bullosa acquisita Atrophic scars and skin fragility on the back of the hands. Courtesy Eliot Mostow, MD.


Epidermolysis bullosa acquisita (EBA) is a rare, autoimmune, subepidermal bullous eruption caused by antibodies to type VII collagen. In vivo, the antibodies bind to the anchoring fibrils of the basement membrane zone.

Clinical

Trauma-induced bulla, milia, and atrophic scars symmetric on dorsa hands, feet and elbows occur in EBA. Some patients may develop mutilating acral involvement with scarring and syndactyly. The disease may be confused clinically with bullous pemphigoid or PCT. Hemorragic bulla are more likely to occur in EBA compared with PCT but this is not always true.

Clinical Presentations of EBA

Variant Clinical Features
Classic Trauma-induced bulla, milia, and atrophic scars symmetric on dorsa hands, feet and elbows
Inflammatory Resembles bullous pemphigoid. Tense bulla with an inflammatory base. Pruritus common.
Cicatricial The cicatricial form involves mucous membranes
Brunsting-Perry The Brunsting-Perry form typically involves the head and neck
Eythroderma Rarely, EBA may present as erythroderma.

Diagnosis

The patient's serum characteristically reacts to the dermal side of salt split skin (just below the lamina densa). It reacts to the 290 kd antigen of anchoring fibrils, and on immune electron microscopy, shows immune deposits over the dermal ends of anchoring fibrils.

Treatment

Treatment may be difficult. Trauma should be reduced.

The following has been recommended [BJD 2014;171;1022]: an initial prednisone dosage below 0·5 mg/kg combined with colchicine 1–2 mg/day and/or dapsone 25–100 mg/day. Prednisone dosage can be gradually tapered once disease control is achieved. In refractory cases, IVIG or rituximab should be considered.

Rituximab

Rituximab has been used with success in both adults and children. Of the 12 adult cases of EBA treated with rituximab, there was one death reported due to Pseudomonas pneumonia.

Additional Pictures

Courtesy Eliot Mostow, MD.
Epidermolysis bullosa acquisita

References

Erythrodermic EBA. JAMA Derm 2015;151;679

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