Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin alpha 3
| Autor: | J van der Meer, Marcel F. Jonkman, F Dijk, Hendrikus Pas, Kim B. Yancey, J Schuur, K Heeres, Marcelus C.J.M. de Jong |
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| Přispěvatelé: | Translational Immunology Groningen (TRIGR) |
| Jazyk: | angličtina |
| Rok vydání: | 2000 |
| Předmět: |
Epidermolysis bullosa acquisita
Pathology medicine.medical_specialty AUTO-ANTIGEN Immunoelectron microscopy INDIRECT IMMUNOFLUORESCENCE Immunoblotting AUTOIMMUNE Dermatology Epidermolysis Bullosa Acquisita DISEASE Diagnosis Differential Laminin Sublamina densa parasitic diseases medicine Humans Cicatricial pemphigoid skin and connective tissue diseases Microscopy Immunoelectron Autoantibodies Basement membrane Inflammation Mucous Membrane EPILIGRIN integumentary system biology IDENTIFICATION business.industry General Medicine Middle Aged medicine.disease Precipitin Tests medicine.anatomical_structure Microscopy Fluorescence Immunoglobulin G Immunology BASEMENT-MEMBRANE ANTIBODIES biology.protein CHAIN Female Epidermolysis bullosa Bullous pemphigoid Collagen business Facial Dermatoses SKIN |
| Zdroj: | Scopus-Elsevier ARCHIVES OF DERMATOLOGY, 136(2), 227-231 |
| ISSN: | 0003-987X |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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