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[Epidermolysis bullosa acquisita].
Presse Med. 2010 Oct; 39(10):1081-8.PM

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal bullous disease with autoimmunity to the type VII collagen which is the major component of anchoring fibrils. Clinical manifestations of the classical EBA include skin fragility, blisters over the trauma-prone surfaces and milium cysts. Other presentations of EBA have been reported: mucosal predominant appearance reminiscent of cicatricial pemphigoid, bullous pemphigoid-like presentation and IgA-EBA. Making a definitive diagnosis of EBA could be difficult because specialized tests available in only some laboratories are necessary to confirm the clinical suspicion: immunoelectron microscopy demonstrating immune deposits on anchoring fibrils and immunoblotting or enzyme-linked immunosorbent assay (Elisa) detecting autoantibodies recognizing the type VII collagen. EBA frequently is associated with Crohn's disease and an inflammatory bowel disease must be ruled out in patients with EBA and abdominal manifestations. EBA potentially is serious, has usually a chronical evolution and is difficult to treat.There are no guidelines for treatment of EBA, which is adapted to clinical severity and include dapsone, cyclosporine and rituximab.

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Authors+Show Affiliations

Caux F
Service de dermatologie et centre de référence des dermatoses bulleuses toxiques et auto-immunes, hôpital Avicenne, AP-HP, 125, rue de Stalingrad, 93000 Bobigny, France. frederic.caux@avc.aphp.fr

MeSH

Antibodies, MonoclonalAntibodies, Monoclonal, Murine-DerivedAutoimmune DiseasesCollagen Type VIICrohn DiseaseCyclosporineDapsoneDiagnosis, DifferentialEnzyme-Linked Immunosorbent AssayEpidermolysis Bullosa AcquisitaFluorescent Antibody TechniqueHistological TechniquesHumansImmunoblottingImmunoglobulin AImmunologic FactorsMicroscopy, ImmunoelectronPractice Guidelines as TopicRare DiseasesRituximabSeverity of Illness Index

Pub Type(s)

English Abstract
Journal Article

Language

fre

PubMed ID

20674256

Citation

Caux, Frédéric. "[Epidermolysis Bullosa Acquisita]." Presse Medicale (Paris, France : 1983), vol. 39, no. 10, 2010, pp. 1081-8.
Caux F. [Epidermolysis bullosa acquisita]. Presse Med. 2010;39(10):1081-8.
Caux, F. (2010). [Epidermolysis bullosa acquisita]. Presse Medicale (Paris, France : 1983), 39(10), 1081-8. https://doi.org/10.1016/j.lpm.2010.04.021
Caux F. [Epidermolysis Bullosa Acquisita]. Presse Med. 2010;39(10):1081-8. PubMed PMID: 20674256.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Epidermolysis bullosa acquisita]. A1 - Caux,Frédéric, Y1 - 2010/08/01/ PY - 2010/04/06/received PY - 2010/04/21/accepted PY - 2010/8/3/entrez PY - 2010/8/3/pubmed PY - 2010/11/3/medline SP - 1081 EP - 8 JF - Presse medicale (Paris, France : 1983) JO - Presse Med VL - 39 IS - 10 N2 - Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal bullous disease with autoimmunity to the type VII collagen which is the major component of anchoring fibrils. Clinical manifestations of the classical EBA include skin fragility, blisters over the trauma-prone surfaces and milium cysts. Other presentations of EBA have been reported: mucosal predominant appearance reminiscent of cicatricial pemphigoid, bullous pemphigoid-like presentation and IgA-EBA. Making a definitive diagnosis of EBA could be difficult because specialized tests available in only some laboratories are necessary to confirm the clinical suspicion: immunoelectron microscopy demonstrating immune deposits on anchoring fibrils and immunoblotting or enzyme-linked immunosorbent assay (Elisa) detecting autoantibodies recognizing the type VII collagen. EBA frequently is associated with Crohn's disease and an inflammatory bowel disease must be ruled out in patients with EBA and abdominal manifestations. EBA potentially is serious, has usually a chronical evolution and is difficult to treat.There are no guidelines for treatment of EBA, which is adapted to clinical severity and include dapsone, cyclosporine and rituximab. SN - 2213-0276 UR - https://www.unboundmedicine.com/medline/citation/20674256/[Epidermolysis_bullosa_acquisita]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0755-4982(10)00419-7 DB - PRIME DP - Unbound Medicine ER -