Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10400.17/4714 |
Resumo: | Background/purpose: Hailey-Hailey disease is a rare inherited acantholytic skin disorder characterized by heterogeneous clinical presentation. Its differential diagnosis might be wide, including other genodermatoses, inflammatory, and infectious skin diseases. Although histopathology remains as diagnostic gold standard, noninvasive techniques such as dermoscopy and reflectance confocal microscopy may assist clinical examination. Herein, we aim to further characterize the dermoscopic and reflectance confocal microscopic presentation of Hailey-Hailey disease with histologic correlation. Methods: Eight patients with Hailey-Hailey disease were consecutively recruited. All patients were examined using dermoscopy and reflectance confocal microscopy. Results: In all cases, dermoscopy enabled the visualization of polymorphous vessels, including glomerular and linear-looped vessels, within a pink-whitish background. Reflectance confocal microscopy revealed wide suprabasilar partial acantholysis and clefting, crusts, dilated papillae with tortuous vessels, and inflammatory cells. Dyskeratosis, uplocated papillae, and adnexal sparing were also observed. Conclusion: Although definite diagnosis was obtained by histopathology in all cases, dermoscopy and reflectance confocal microscopy allowed the identification of common features (even in cases with dissimilar clinical presentation) that may support an early diagnosis of Hailey-Hailey disease, and its differentiation from other more frequent skin disorders. |
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Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case SeriesHSAC DERHumansAdultMaleFemaleMiddle AgedDermoscopy / methodsDiagnosis, DifferentialMicroscopy, Confocal / methodsPemphigus, Benign Familial / diagnosis*Pemphigus, Benign Familial / pathologyBackground/purpose: Hailey-Hailey disease is a rare inherited acantholytic skin disorder characterized by heterogeneous clinical presentation. Its differential diagnosis might be wide, including other genodermatoses, inflammatory, and infectious skin diseases. Although histopathology remains as diagnostic gold standard, noninvasive techniques such as dermoscopy and reflectance confocal microscopy may assist clinical examination. Herein, we aim to further characterize the dermoscopic and reflectance confocal microscopic presentation of Hailey-Hailey disease with histologic correlation. Methods: Eight patients with Hailey-Hailey disease were consecutively recruited. All patients were examined using dermoscopy and reflectance confocal microscopy. Results: In all cases, dermoscopy enabled the visualization of polymorphous vessels, including glomerular and linear-looped vessels, within a pink-whitish background. Reflectance confocal microscopy revealed wide suprabasilar partial acantholysis and clefting, crusts, dilated papillae with tortuous vessels, and inflammatory cells. Dyskeratosis, uplocated papillae, and adnexal sparing were also observed. Conclusion: Although definite diagnosis was obtained by histopathology in all cases, dermoscopy and reflectance confocal microscopy allowed the identification of common features (even in cases with dissimilar clinical presentation) that may support an early diagnosis of Hailey-Hailey disease, and its differentiation from other more frequent skin disorders.WileyRepositório do Centro Hospitalar Universitário de Lisboa Central, EPEOliveira, AArzberger, EPimentel, BSousa, VLeal‐Filipe, P2023-10-09T14:22:02Z2017-022017-02-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.17/4714engSkin Res Technol . 2018 Feb;24(1):85-92.10.1111/srt.12394info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-10-15T06:33:07Zoai:repositorio.chlc.min-saude.pt:10400.17/4714Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:35:47.031857Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series |
title |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series |
spellingShingle |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series Oliveira, A HSAC DER Humans Adult Male Female Middle Aged Dermoscopy / methods Diagnosis, Differential Microscopy, Confocal / methods Pemphigus, Benign Familial / diagnosis* Pemphigus, Benign Familial / pathology |
title_short |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series |
title_full |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series |
title_fullStr |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series |
title_full_unstemmed |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series |
title_sort |
Dermoscopic and Reflectance Confocal Microscopic Presentation of Hailey‐Hailey Disease: a Case Series |
author |
Oliveira, A |
author_facet |
Oliveira, A Arzberger, E Pimentel, B Sousa, V Leal‐Filipe, P |
author_role |
author |
author2 |
Arzberger, E Pimentel, B Sousa, V Leal‐Filipe, P |
author2_role |
author author author author |
dc.contributor.none.fl_str_mv |
Repositório do Centro Hospitalar Universitário de Lisboa Central, EPE |
dc.contributor.author.fl_str_mv |
Oliveira, A Arzberger, E Pimentel, B Sousa, V Leal‐Filipe, P |
dc.subject.por.fl_str_mv |
HSAC DER Humans Adult Male Female Middle Aged Dermoscopy / methods Diagnosis, Differential Microscopy, Confocal / methods Pemphigus, Benign Familial / diagnosis* Pemphigus, Benign Familial / pathology |
topic |
HSAC DER Humans Adult Male Female Middle Aged Dermoscopy / methods Diagnosis, Differential Microscopy, Confocal / methods Pemphigus, Benign Familial / diagnosis* Pemphigus, Benign Familial / pathology |
description |
Background/purpose: Hailey-Hailey disease is a rare inherited acantholytic skin disorder characterized by heterogeneous clinical presentation. Its differential diagnosis might be wide, including other genodermatoses, inflammatory, and infectious skin diseases. Although histopathology remains as diagnostic gold standard, noninvasive techniques such as dermoscopy and reflectance confocal microscopy may assist clinical examination. Herein, we aim to further characterize the dermoscopic and reflectance confocal microscopic presentation of Hailey-Hailey disease with histologic correlation. Methods: Eight patients with Hailey-Hailey disease were consecutively recruited. All patients were examined using dermoscopy and reflectance confocal microscopy. Results: In all cases, dermoscopy enabled the visualization of polymorphous vessels, including glomerular and linear-looped vessels, within a pink-whitish background. Reflectance confocal microscopy revealed wide suprabasilar partial acantholysis and clefting, crusts, dilated papillae with tortuous vessels, and inflammatory cells. Dyskeratosis, uplocated papillae, and adnexal sparing were also observed. Conclusion: Although definite diagnosis was obtained by histopathology in all cases, dermoscopy and reflectance confocal microscopy allowed the identification of common features (even in cases with dissimilar clinical presentation) that may support an early diagnosis of Hailey-Hailey disease, and its differentiation from other more frequent skin disorders. |
publishDate |
2017 |
dc.date.none.fl_str_mv |
2017-02 2017-02-01T00:00:00Z 2023-10-09T14:22:02Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10400.17/4714 |
url |
http://hdl.handle.net/10400.17/4714 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Skin Res Technol . 2018 Feb;24(1):85-92. 10.1111/srt.12394 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Wiley |
publisher.none.fl_str_mv |
Wiley |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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