Abstract
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, immuno-blot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin alpha 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.
| Original language | English |
|---|---|
| Pages (from-to) | 227-231 |
| Number of pages | 5 |
| Journal | Archives of Dermatology |
| Volume | 136 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - Feb-2000 |
| Event | Joint Society Meeting of the European-Academy-of-Dermatology-and-Venereology/Groupe Immunodermatologie, Societe-Francaise-de-Dermatologie - , France Duration: 7-Oct-1998 → … |
Keywords
- BASEMENT-MEMBRANE
- INDIRECT IMMUNOFLUORESCENCE
- AUTO-ANTIGEN
- SKIN
- AUTOIMMUNE
- ANTIBODIES
- EPILIGRIN
- DISEASE
- CHAIN
- IDENTIFICATION