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Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review

Pemphigoid nodularis (PN) is a rare clinical variant of bullous pemphigoid characterized by the presence of nodular prurigo-like lesions and pemphigoid blisters. The diagnosis is confirmed by direct immunofluorescence (DIF)/ indirect immunofluorescence (IIF) and immunoserology tests. For some patien...

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Autores principales: Vornicescu, Corina, Șenilă, Simona Corina, Cosgarea, Rodica, Candrea, Elisabeta, Pop, Alexandra Dana, Ungureanu, Loredana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: D.A. Spandidos 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327548/
https://www.ncbi.nlm.nih.gov/pubmed/30679985
http://dx.doi.org/10.3892/etm.2018.7057
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author Vornicescu, Corina
Șenilă, Simona Corina
Cosgarea, Rodica
Candrea, Elisabeta
Pop, Alexandra Dana
Ungureanu, Loredana
author_facet Vornicescu, Corina
Șenilă, Simona Corina
Cosgarea, Rodica
Candrea, Elisabeta
Pop, Alexandra Dana
Ungureanu, Loredana
author_sort Vornicescu, Corina
collection PubMed
description Pemphigoid nodularis (PN) is a rare clinical variant of bullous pemphigoid characterized by the presence of nodular prurigo-like lesions and pemphigoid blisters. The diagnosis is confirmed by direct immunofluorescence (DIF)/ indirect immunofluorescence (IIF) and immunoserology tests. For some patients, with long mean duration of symptoms, the correct diagnosis of PN is delayed because the disease is not recognized. We present a case and summarize the reported characteristics of PN. The search in MEDLINE database, after selection, resulted in 36 articles presenting 47 cases of PN. Between published cases a female predominance was noted (female to male ratio of 1.8:1), almost half of the reported patients were non-Caucasian, and the mean age at presentation was 66.2 years. The mean duration until the diagnosis was almost 2 years. Sixteen patients also had other autoimmune diseases. Twenty-two patients developed vesicles/bullae/urticarial plaques before or after the diagnosis. Peripheral eosinophilia and high levels of serum total IgE were reported in 10.6 and 27.2% of patients, respectively. ELISA for either BP180, BP230 or both were positive in all tested cases. DIF and IIF microscopy were positive overall in 100 and 92.3% of cases, respectively. Corticosteroids, either topical or systemic, were the most efficient therapeutic option, although many others were used. PN remains a diagnostic and therapeutic challenge in elderly patients with unexplained refractory chronic pruritus associated with papulo-nodular lesions.
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spelling pubmed-63275482019-01-24 Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review Vornicescu, Corina Șenilă, Simona Corina Cosgarea, Rodica Candrea, Elisabeta Pop, Alexandra Dana Ungureanu, Loredana Exp Ther Med Articles Pemphigoid nodularis (PN) is a rare clinical variant of bullous pemphigoid characterized by the presence of nodular prurigo-like lesions and pemphigoid blisters. The diagnosis is confirmed by direct immunofluorescence (DIF)/ indirect immunofluorescence (IIF) and immunoserology tests. For some patients, with long mean duration of symptoms, the correct diagnosis of PN is delayed because the disease is not recognized. We present a case and summarize the reported characteristics of PN. The search in MEDLINE database, after selection, resulted in 36 articles presenting 47 cases of PN. Between published cases a female predominance was noted (female to male ratio of 1.8:1), almost half of the reported patients were non-Caucasian, and the mean age at presentation was 66.2 years. The mean duration until the diagnosis was almost 2 years. Sixteen patients also had other autoimmune diseases. Twenty-two patients developed vesicles/bullae/urticarial plaques before or after the diagnosis. Peripheral eosinophilia and high levels of serum total IgE were reported in 10.6 and 27.2% of patients, respectively. ELISA for either BP180, BP230 or both were positive in all tested cases. DIF and IIF microscopy were positive overall in 100 and 92.3% of cases, respectively. Corticosteroids, either topical or systemic, were the most efficient therapeutic option, although many others were used. PN remains a diagnostic and therapeutic challenge in elderly patients with unexplained refractory chronic pruritus associated with papulo-nodular lesions. D.A. Spandidos 2019-02 2018-12-05 /pmc/articles/PMC6327548/ /pubmed/30679985 http://dx.doi.org/10.3892/etm.2018.7057 Text en Copyright: © Vornicescu et al. This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
spellingShingle Articles
Vornicescu, Corina
Șenilă, Simona Corina
Cosgarea, Rodica
Candrea, Elisabeta
Pop, Alexandra Dana
Ungureanu, Loredana
Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review
title Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review
title_full Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review
title_fullStr Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review
title_full_unstemmed Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review
title_short Pemphigoid nodularis - rare presentation of bullous pemphigoid: A case report and literature review
title_sort pemphigoid nodularis - rare presentation of bullous pemphigoid: a case report and literature review
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327548/
https://www.ncbi.nlm.nih.gov/pubmed/30679985
http://dx.doi.org/10.3892/etm.2018.7057
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