Track: Case Reports

Abstract:

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal blistering disorder caused by autoantibodies against type VII collagen. Its occurrence after bone marrow transplantation (BMT) is uncommon and may resemble graft-versus-host disease (GVHD) or other autoimmune blistering diseases, both clinically and histologically.

A 30-year-old woman with a history of idiopathic aplastic anemia and subsequent allogeneic BMT at age 17. She presented with a one-month history of painful, pruritic vesicles. Prior to dermatology assessment, she had been treated with topical and systemic corticosteroids, antibiotics, antifungals, and antivirals without sustained improvement. Examination showed dozens of vesicles and erosions on the dorsal hands, face, scalp, neck, back, arms, feet, and involvement of the vermilion and mucosal lips. .

Histologic examination revealed a subepidermal blister with neutrophils, eosinophils, and scattered dyskeratotic keratinocytes. Direct immunofluorescence showed strong linear basement membrane deposition of IgG and IgM with weak C3. Indirect immunofluorescence on salt-split skin demonstrated IgG deposition on the dermal side. Serologic studies were positive for anti–type VII collagen antibodies and negative for BP180, BP230, ANA, and anti-dsDNA, making bullous lupus erythematosus unlikely.

The overall findings support a diagnosis of EBA in the post-bone marrow transplant setting. The patient showed partial improvement with colchicine, with resolution of the mucosal lesions. Dapsone therapy is planned pending G6PD testing.

This case highlights recognition of EBA as a rare post-transplant autoimmune dermatosis and underscores the importance of thorough clinicopathologic and serologic correlation in evaluating vesiculobullous eruptions after BMT.

Tessa Farnham

Affiliation

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(538) Late Development of Epidermolysis Bullosa Acquisita in a Bone Marrow Transplant Recipient