Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin alpha 3

Background: The inflammatory variant of epidermolysis bullosa acquisita (EB A) may clinically closely resemble bullous or cicatricial pemphigoid. Patie nts with inflammatory EBA have IgG autoantibodies against type VII collagen . Patients with anti-epiligrin cicatricial pemphigoid have IgG autoantibodi es against laminin 5. Observation: We describe a patient with inflammatory EBA exhibiting nonscar ring oral and vaginal involvement. Indirect immunofluorescence using skin s ubstrate lacking an epidermal basement membrane molecule, direct immunoelec tron microscopy, immuno-blot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII co llagen and laminin alpha 3. A final diagnosis of EBA was based on the subla mina densa level of blister formation. Conclusion: This case illustrates the clinical and immunological overlap be tween EBA and anti-epiligrin cicatricial pemphigoid, a unique finding that may have developed as a consequence of epitope spreading.