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Inflammatory Variant of Epidermolysis Bullosa Acquisita With IgG Autoantibodies Against Type VII Collagen and Laminin  3
Marcel F. Jonkman, MD, PhD;
Jacqueline Schuur, MD;
Freark Dijk;
Klaas Heeres;
Marcelus C. J. M. de Jong, PhD;
Jan B. van der Meer, MD, PhD;
Kim B. Yancey, MD;
Hendri H. Pas, PhD
Arch Dermatol. 2000;136:227-231.
Background The inflammatory variant of epidermolysis bullosa acquisita (EBA) may clinically closely resemble bullous or cicatricial pemphigoid. Patients with inflammatory EBA have IgG autoantibodies against type VII collagen. Patients with anti–epiligrin cicatricial pemphigoid have IgG autoantibodies against laminin 5.
Observation We describe a patient with inflammatory EBA exhibiting nonscarring oral and vaginal involvement. Indirect immunofluorescence using skin substrate lacking an epidermal basement membrane molecule, direct immunoelectron microscopy, immunoblot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin  3. A final diagnosis of EBA was based on the sublamina densa level of blister formation.
Conclusion This case illustrates the clinical and immunological overlap between EBA and anti–epiligrin cicatricial pemphigoid, a unique finding that may have developed as a consequence of epitope spreading.
From the Department of Dermatology (Drs Jonkman, Schuur, de Jong, van der Meer, and Pas and Mr Heeres) and the Laboratory for Cell Biology and Electron Microscopy (Mr Dijk), University of Groningen, Groningen, the Netherlands; and the Dermatology Branch, National Career Institute, National Institutes of Health, Bethesda, Md (Dr Yancey).
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