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Retrospective study of the clinical, histologic, and immunologic features of epidermolysis bullosa acquisita in 9 patients.
Actas Dermosifiliogr. 2013 Dec; 104(10):904-14.AD

Abstract

INTRODUCTION

Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease caused by autoantibodies to type VII collagen. The clinical presentation is variable, with skin and mucosal lesions that can cause significant dysfunction. Different treatment options exist, but the results are often unsatisfactory.

OBJECTIVE

To review all the cases of epidermolysis bullosa acquisita (EBA) diagnosed at our hospital over a 26-year period.

MATERIALS AND METHODS

We performed a retrospective review of the clinical, histologic, and immunologic features of EBA in 9 patients.

RESULTS

Mean age at presentation was 37 years and 66.67% of the patients were women. EBA occurred in association with malignant tumors, inflammatory bowel disease, and autoimmune disorders. The most common variant was inflammatory EBA (6 of the 9 cases). In all 9 patients, histology revealed a subepidermal blister and direct immunofluorescence showed linear deposits of immunoglobulin G and C3 in the basement membrane zone. Indirect immunofluorescence performed on salt-split skin substrate was positive in 6 patients and showed a dermal pattern in all cases. Five patients were tested for autoantibodies to type VII collagen using enzyme-linked immunosorbent assay, with positive results in 2 cases. Immunoblotting using recombinant noncollagenous domains (NC1) of type VII collagen was positive in all 6 cases in which it was performed. Response to treatment was variable.

CONCLUSIONS

EBA is a rare disease with a variable clinical presentation that can be confused with that of other subepidermal blistering diseases. Correct diagnosis requires a high level of clinical suspicion and the use of all available diagnostic tests. Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications.

Authors+Show Affiliations

Servicio de Dermatología, Hospital Clínic, Universitat de Barcelona, Barcelona, España.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng spa

PubMed ID

23895729

Citation

Barreiro-Capurro, A, et al. "Retrospective Study of the Clinical, Histologic, and Immunologic Features of Epidermolysis Bullosa Acquisita in 9 Patients." Actas Dermo-sifiliograficas, vol. 104, no. 10, 2013, pp. 904-14.
Barreiro-Capurro A, Mascaró-Galy JM, Iranzo P. Retrospective study of the clinical, histologic, and immunologic features of epidermolysis bullosa acquisita in 9 patients. Actas Dermosifiliogr. 2013;104(10):904-14.
Barreiro-Capurro, A., Mascaró-Galy, J. M., & Iranzo, P. (2013). Retrospective study of the clinical, histologic, and immunologic features of epidermolysis bullosa acquisita in 9 patients. Actas Dermo-sifiliograficas, 104(10), 904-14. https://doi.org/10.1016/j.ad.2013.05.005
Barreiro-Capurro A, Mascaró-Galy JM, Iranzo P. Retrospective Study of the Clinical, Histologic, and Immunologic Features of Epidermolysis Bullosa Acquisita in 9 Patients. Actas Dermosifiliogr. 2013;104(10):904-14. PubMed PMID: 23895729.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Retrospective study of the clinical, histologic, and immunologic features of epidermolysis bullosa acquisita in 9 patients. AU - Barreiro-Capurro,A, AU - Mascaró-Galy,J M, AU - Iranzo,P, Y1 - 2013/07/26/ PY - 2012/12/23/received PY - 2013/04/30/revised PY - 2013/05/25/accepted PY - 2013/7/31/entrez PY - 2013/7/31/pubmed PY - 2015/1/28/medline KW - Autoimmune subepidermal bullous disease KW - Enfermedad ampollosa subepidérmica autoinmune KW - Epidermolysis bullosa acquisita KW - Epidermólisis ampollosa adquirida SP - 904 EP - 14 JF - Actas dermo-sifiliograficas JO - Actas Dermosifiliogr VL - 104 IS - 10 N2 - INTRODUCTION: Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease caused by autoantibodies to type VII collagen. The clinical presentation is variable, with skin and mucosal lesions that can cause significant dysfunction. Different treatment options exist, but the results are often unsatisfactory. OBJECTIVE: To review all the cases of epidermolysis bullosa acquisita (EBA) diagnosed at our hospital over a 26-year period. MATERIALS AND METHODS: We performed a retrospective review of the clinical, histologic, and immunologic features of EBA in 9 patients. RESULTS: Mean age at presentation was 37 years and 66.67% of the patients were women. EBA occurred in association with malignant tumors, inflammatory bowel disease, and autoimmune disorders. The most common variant was inflammatory EBA (6 of the 9 cases). In all 9 patients, histology revealed a subepidermal blister and direct immunofluorescence showed linear deposits of immunoglobulin G and C3 in the basement membrane zone. Indirect immunofluorescence performed on salt-split skin substrate was positive in 6 patients and showed a dermal pattern in all cases. Five patients were tested for autoantibodies to type VII collagen using enzyme-linked immunosorbent assay, with positive results in 2 cases. Immunoblotting using recombinant noncollagenous domains (NC1) of type VII collagen was positive in all 6 cases in which it was performed. Response to treatment was variable. CONCLUSIONS: EBA is a rare disease with a variable clinical presentation that can be confused with that of other subepidermal blistering diseases. Correct diagnosis requires a high level of clinical suspicion and the use of all available diagnostic tests. Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications. SN - 1578-2190 UR - https://www.unboundmedicine.com/medline/citation/23895729/Retrospective_study_of_the_clinical_histologic_and_immunologic_features_of_epidermolysis_bullosa_acquisita_in_9_patients_ L2 - https://www.elsevier.es/en/linksolver/pdf/pii/S0001-7310(13)00252-4 DB - PRIME DP - Unbound Medicine ER -